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		<id>https://www.wikidoc.org/index.php?title=Leprosy_differential_diagnosis&amp;diff=1071730</id>
		<title>Leprosy differential diagnosis</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Leprosy_differential_diagnosis&amp;diff=1071730"/>
		<updated>2015-02-20T16:14:17Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Differential diagnosis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Leprosy}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
Leprosy must be differentiated from other diseases that cause [[skin lesions]], [[nodules]], [[plaques]]  [[paresthesias]] and [[nerve pain]], such as [[autoimmune diseases]], [[SLE]], [[parasitic infections]], [[vitiligo]] or [[tuberculosis|cutaneous tuberculosis]].&lt;br /&gt;
&lt;br /&gt;
==Differential diagnosis==&lt;br /&gt;
Leprosy has a wide range of clinical manifestations, each with different degrees of intensity. Other [[diseases]] that may mimic, or have similar features of leprosy, include:&amp;lt;ref name=&amp;quot;WalkerLockwood2007&amp;quot;&amp;gt;{{cite journal|last1=Walker|first1=Stephen L.|last2=Lockwood|first2=Dina N.J.|title=Leprosy|journal=Clinics in Dermatology|volume=25|issue=2|year=2007|pages=165–172|issn=0738081X|doi=10.1016/j.clindermatol.2006.05.012}}&amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Congenital disorder|Congenital lesions]]&#039;&#039;&#039; - such as [[nevus depigmentosus]] are generally present at birth and do not present with changes in [[sensation]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Vitiligo]]&#039;&#039;&#039; - a [[benign]] lesion that presents with depigmented areas, instead of [[hypopigmentation|hypopigmented]]. &lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Pityriasis alba]]&#039;&#039;&#039; - may be hard to distinguish from leprosy, particularly in early stages of the second, however, it is a common [[skin]] condition, mostly occurring in children and usually seen as dry, fine scaled, pale patches on their faces.&amp;lt;ref name=&amp;quot;Pinto&amp;quot;&amp;gt;{{cite journal |author=Pinto FJ, Bolognia JL |title=Disorders of hypopigmentation in children |journal=Pediatr. Clin. North Am. |volume=38 |issue=4 |pages=991-1017 |year=1991 |pmid=1870914}}&amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Pityriasis versicolor]]&#039;&#039;&#039; - common [[skin]] [[infection]] caused by the yeast &#039;&#039;[[Malassezia furfur]]&#039;&#039;. This [[yeast]] is normally found on the human [[skin]] and only becomes troublesome under certain circumstances, such as a warm and humid environment, causing: generally oval or irregularly-shaped spots of 1/4 to 1 inch in diameter, often merging together to form a larger patch with a sharp border; occasional fine scaling of the [[skin]] producing a very superficial ash-like scale; and pale, dark tan, or pink in color spots, with a reddish undertone that can darken when the patient is overheated, such as in a hot shower or during/after exercise.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Dermatophyte]]&#039;&#039;&#039; - [[parasitic]] [[fungus]] that [[infection|infects]] the the [[skin]], [[hair]] and nails due to its ability to obtain [[nutrients]] from [[keratinized]] material. The infection is usually restricted to the nonliving cornified layer of the [[epidermis]] because of their inability to penetrate viable tissue of an [[immunocompetent]] host.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Systemic Lupus Erythematosus]]&#039;&#039;&#039; - potential fatal [[chronic]] [[autoimmune disease]], common initial and [[chronic]] complaints include [[skin rash]], [[fever]], [[malaise]], [[arthralgia|joint pains]], [[myalgia]]s and [[fatigue]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Postinflammatory hypopigmentation|Postinflammatory hypopigmented]] skin&#039;&#039;&#039; -  a [[cutaneous]] condition characterized by decreased [[pigment]] in the [[skin]] following [[inflammation|inflammatory reaction]] of the [[skin]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Granuloma multiforme]]&#039;&#039;&#039; - a [[cutaneous]] condition most commonly seen in central Africa, and rarely elsewhere, characterized by [[skin lesion]]s that are located predominantly on the [[upper trunk]] and [[arm]]s in sun-exposed areas.&amp;lt;ref name=&amp;quot;Andrews&amp;quot;&amp;gt;{{cite book |author=James, William D.; Berger, Timothy G.; et al. |title=Andrews&#039; Diseases of the Skin: clinical Dermatology |publisher=Saunders Elsevier |location= |year=2006 |pages= |isbn=0-7216-2921-0 |oclc= |doi= |accessdate=}}&amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Cutaneous]] [[tuberculosis]]&#039;&#039;&#039; - a form of [[tuberculosis]] involving the [[skin]]. The [[diagnosis]] may be difficult, since this condition shares many similarities with other [[skin]] conditions. It may be caused by [[Mycobacterium tuberculosis]], [[Mycobacterium bovis]], and the [[BCG]] [[vaccination]]. Commonly the [[skin lesion]] begins as an irregular red-brown [[papule]] or [[nodule]], with a [[granulomatous]] base. Later this lesion evolves into a painless [[ulcer]], usually with ≤ 1cm of diameter. The most common sites of this [[lesion]] are: [[face]] and [[extremities]].&amp;lt;ref name=&amp;quot;pmid8521366&amp;quot;&amp;gt;{{cite journal| author=MacGregor RR| title=Cutaneous tuberculosis. | journal=Clin Dermatol | year= 1995 | volume= 13 | issue= 3 | pages= 245-55 | pmid=8521366 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8521366  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Granuloma annulare]]&#039;&#039;&#039; - [[chronic]] [[skin disease]], with unknown cause, consisting of a [[rash]] with reddish bumps arranged in a circle or ring. It most often affects [[children]], young and older [[adults]] and it is slightly more common in [[females]] (60/40 ratio). The condition is usually seen in otherwise healthy people. Occasionally, it may be associated with [[diabetes]] or [[thyroid disease]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Cutaneous leishmaniasis]]&#039;&#039;&#039; - a [[skin infection]] caused by a single-celled [[parasite]] that is transmitted by sand-fly bites the, constituting the most common form of [[leishmaniasis]]. The lesions typically progress from small [[papules]] to [[nodular]] [[plaques]], and eventually lead to open sores with a raised border and central crater ([[ulcer]]), which can be covered with scales or [[crust]. These [[lesions]] are usually painless but can be painful, particularly if open sores become [[infected]] with [[bacteria]]. Satellite lesions, regional [[lymphadenopathy]] (swollen [[glands]]), and [[nodular]] [[lymphangitis]] can be noted. Commonly this condition produces less nodules than the lepromatous form of leprosy, with the [[skin lesions]] commonly resolving after a few weeks, which is uncommon in leprosy.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Post kala-azar dermal leishmaniasis]]&#039;&#039;&#039; - may mimic leprosy in its presentation with hypopigmented [[macules]], [[papules]] and [[nodular lesions]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Mycosis fungoides]]&#039;&#039;&#039; - is a rare form of [[cutaneous]] [[T-cell]] [[non-Hodgkin&#039;s lymphoma]], generally affects the [[skin]], but may progress internally over time. Common [[symptoms]] include [[rash|rashlike]] patches, [[tumors]], or [[lesions]] and [[pruritus]] in about 20% of patients.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Hereditary]] [[sensory neuron|sensory]] [[motor neuron]] [[neuropathy]] type III&#039;&#039;&#039; - commonly causes [[nerve thickening]], which is present in leprosy and may therefore complicate the [[diagnosis]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Refsum&#039;s disease]]&#039;&#039;&#039; - like  [[hereditary]] [[sensory neuron|sensory]] [[motor neuron]] [[neuropathy]] type III, it commonly causes [[nerve thickening]], which is present in leprosy and may therefore complicate the [[diagnosis]].&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Amyloid]]&#039;&#039;&#039; - insoluble fibrous [[protein]] aggregations sharing specific structural traits. Is able to cause a wide spectrum of [[symptoms]], of which [[skin]] changes is a common form of presentation. These include: [[petechiae]], [[ecchymoses]], [[waxy papular [[nodules]] around the [[eyes]], [[neck]] and in the [[groin]] region, patches of [[hair]] loss and [[brittle nails]]. May be a [[complication]] of leprosy and be responsible for part of the [[nerve]] thickening seen in this condition.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Mycosis]]&#039;&#039;&#039; - may be classified according to the affected [[tissue]]. When it affects the superficial and more inner layers of [[skin]] it may mimic leprosy.&lt;br /&gt;
&lt;br /&gt;
* &#039;&#039;&#039;[[Keloid]]&#039;&#039;&#039; - A keloid is a type of scar which results in an overgrowth of tissue at the site of a healed skin injury. Keloids are firm, rubbery lesions or shiny, fibrous nodules, and can vary from pink to flesh-colored or red to dark brown in color.&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Dermatology]]&lt;br /&gt;
[[Category:Infectious disease]]&lt;br /&gt;
[[Category:Tropical disease]]&lt;br /&gt;
[[Category:Leprosy]]&lt;br /&gt;
[[Category:Infectious skin diseases]]&lt;br /&gt;
[[Category:Bacterial diseases]]&lt;br /&gt;
[[Category:Neglected diseases]]&lt;br /&gt;
[[Category:Needs content]]&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Subdural_empyema_lumbar_puncture&amp;diff=1071440</id>
		<title>Subdural empyema lumbar puncture</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Subdural_empyema_lumbar_puncture&amp;diff=1071440"/>
		<updated>2015-02-19T22:21:37Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Subdural empyema}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
Subdural empyema, also referred to as [[subdural abscess]], [[pachymeningitis interna]] and [[circumscript meningitis]], is a life-threatening [[infection]].&amp;lt;ref name=&amp;quot;AgrawalTimothy2007&amp;quot;&amp;gt;{{cite journal|last1=Agrawal|first1=Amit|last2=Timothy|first2=Jake|last3=Pandit|first3=Lekha|last4=Shetty|first4=Lathika|last5=Shetty|first5=J.P.|title=A Review of Subdural Empyema and Its Management|journal=Infectious Diseases in Clinical Practice|volume=15|issue=3|year=2007|pages=149–153|issn=1056-9103|doi=10.1097/01.idc.0000269905.67284.c7}}&amp;lt;/ref&amp;gt; It consists of a localised collection of [[pus|purulent]] material, usually unilateral, between the [[dura mater]] and the [[arachnoid mater]] and accounts for about 15-22% of the reported focal intracranial [[infections]].  The [[empyema]] may develop intracranially (about 95%) or in the [[spinal canal]] (about 5%), and in both cases, it constitutes a [[medical emergency|medical]] and [[surgical emergency|neurosurgical emergency]].&amp;lt;ref name=&amp;quot;pmid12521560&amp;quot;&amp;gt;{{cite journal| author=Greenlee JE| title=Subdural Empyema. | journal=Curr Treat Options Neurol | year= 2003 | volume= 5 | issue= 1 | pages= 13-22 | pmid=12521560 | doi= | pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=12521560  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
The [[Lumbar Puncture]] is an invasive procedure which is contraindicated in case of suspicion of subdural empyema and [[increased intracranial pressure]], due to risk of [[brain herniation]] and death.&amp;lt;ref name=&amp;quot;pmid12521560&amp;quot;&amp;gt;{{cite journal| author=Greenlee JE| title=Subdural Empyema. | journal=Curr Treat Options Neurol | year= 2003 | volume= 5 | issue= 1 | pages= 13-22 | pmid=12521560 | doi= | pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=12521560  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Lumbar Puncture==&lt;br /&gt;
In&#039;&#039;&#039;infants&#039;&#039;&#039;, in which subdural empyema is most commonly a complication of [[meningitis]], [[spinal fluid]] culture is the gold-stadard test for the diagnosis, therefore it should be used after ruling-out [[increased intracranial pressure]] &amp;lt;ref name=&amp;quot;Hendaus2013&amp;quot;&amp;gt;{{cite journal|last1=Hendaus|first1=Mohammed A.|title=Subdural Empyema in Children|journal=Global Journal of Health Science|volume=5|issue=6|year=2013|issn=1916-9744|doi=10.5539/gjhs.v5n6p54}}&amp;lt;/ref&amp;gt;.&lt;br /&gt;
&lt;br /&gt;
In &#039;&#039;&#039;adults&#039;&#039;&#039;, in which subdural empyema is most commonly a complication of [[sinusitis]], [[spinal fluid]] in normal circumstances is sterile, however, changes in [[white blood cell count]], [[glucose]] and [[protein]] concentrations are usually unspecific. Nevertheless, after increased intracranial pressure has been excluded, the [[lumbar puncture]] is a useful diagnostic test to rule out meningeal [[infection]]. &amp;lt;ref name=&amp;quot;AgrawalTimothy2007&amp;quot;&amp;gt;{{cite journal|last1=Agrawal|first1=Amit|last2=Timothy|first2=Jake|last3=Pandit|first3=Lekha|last4=Shetty|first4=Lathika|last5=Shetty|first5=J.P.|title=A Review of Subdural Empyema and Its Management|journal=Infectious Diseases in Clinical Practice|volume=15|issue=3|year=2007|pages=149–153|issn=1056-9103|doi=10.1097/01.idc.0000269905.67284.c7}}&amp;lt;/ref&amp;gt;&lt;br /&gt;
The CSF findings which may suggest an [[infection]] are:&lt;br /&gt;
*increased [[white blood cell count]]&lt;br /&gt;
*increased [[protein]] level&lt;br /&gt;
*decreased [[glucose]] levels&lt;br /&gt;
*occasionally [[CSF]] results may be normal and sterile&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{Reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Primary care]]&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Infectious disease]]&lt;br /&gt;
[[Category:Neurology]]&lt;br /&gt;
[[Category:Emergency medicine]]&lt;br /&gt;
[[Category:Diseases involving the fasciae]]&lt;br /&gt;
[[Category:Inflammations]]&lt;br /&gt;
[[Category:Neurological disorders]]&lt;br /&gt;
&lt;br /&gt;
{{WH}}&lt;br /&gt;
{{WS}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024165</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024165"/>
		<updated>2014-09-19T20:18:38Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
In this form of liposarcoma there is a transition from a low-grade differentiation, to a high-grade differentiation within the same mass of well-differentiated liposarcoma.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid534388&amp;quot;&amp;gt;{{cite journal| author=Evans HL| title=Liposarcoma: a study of 55 cases with a reassessment of its classification. | journal=Am J Surg Pathol | year= 1979 | volume= 3 | issue= 6 | pages= 507-23 | pmid=534388 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=534388  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid192432&amp;quot;&amp;gt;{{cite journal| author=Dahlin DC, Unni KK, Matsuno T| title=Malignant (fibrous) histiocytoma of bone--fact or fancy?. | journal=Cancer | year= 1977 | volume= 39 | issue= 4 | pages= 1508-16 | pmid=192432 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=192432  }} &amp;lt;/ref&amp;gt;  Dedifferentiation from well-differented liposarcomas occurs in 90% of the cases in the primary tumor, and in the remaining in recurrent neoplasms.&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
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===Myxoid Liposarcoma===&lt;br /&gt;
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===Round Cell Liposarcoma===&lt;br /&gt;
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===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
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==Associated Conditions==&lt;br /&gt;
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==Gross Pathology==&lt;br /&gt;
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==Microscopic Pathology==&lt;br /&gt;
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==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024161</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024161"/>
		<updated>2014-09-19T20:11:52Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
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&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
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==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
In this form of liposarcoma there is a transition from a low-grade differentiation, to a high-grade differentiation within the same mass of well-differentiated liposarcoma.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid534388&amp;quot;&amp;gt;{{cite journal| author=Evans HL| title=Liposarcoma: a study of 55 cases with a reassessment of its classification. | journal=Am J Surg Pathol | year= 1979 | volume= 3 | issue= 6 | pages= 507-23 | pmid=534388 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=534388  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid192432&amp;quot;&amp;gt;{{cite journal| author=Dahlin DC, Unni KK, Matsuno T| title=Malignant (fibrous) histiocytoma of bone--fact or fancy?. | journal=Cancer | year= 1977 | volume= 39 | issue= 4 | pages= 1508-16 | pmid=192432 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=192432  }} &amp;lt;/ref&amp;gt;  Dedifferentiation from well-differented liposarcomas occurs in 90% of the cases in the primary tumor, and in the remaining in recurrent neoplasms.&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024154</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024154"/>
		<updated>2014-09-19T20:02:28Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
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&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
In this form of liposarcoma there is a transition from a low-grade differentiation, to a high-grade differentiation within the same mass of well-differentiated liposarcoma.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid534388&amp;quot;&amp;gt;{{cite journal| author=Evans HL| title=Liposarcoma: a study of 55 cases with a reassessment of its classification. | journal=Am J Surg Pathol | year= 1979 | volume= 3 | issue= 6 | pages= 507-23 | pmid=534388 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=534388  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid192432&amp;quot;&amp;gt;{{cite journal| author=Dahlin DC, Unni KK, Matsuno T| title=Malignant (fibrous) histiocytoma of bone--fact or fancy?. | journal=Cancer | year= 1977 | volume= 39 | issue= 4 | pages= 1508-16 | pmid=192432 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=192432  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
First described in 1979 by Evans,22 who coopted the term from Dhalin et al’s23 description of tumor progression in chondrosarcoma, dedifferentiation in well- differentiated liposarcoma tends to occur more fre- quently in the primary tumor (90%) but can also be observed in recurrences (10%). The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
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==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024150</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024150"/>
		<updated>2014-09-19T19:58:58Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
In this form of liposarcoma there is a transition from a low-grade differentiation, to a high-grade differentiation within the same mass of well-differentiated liposarcoma.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid534388&amp;quot;&amp;gt;{{cite journal| author=Evans HL| title=Liposarcoma: a study of 55 cases with a reassessment of its classification. | journal=Am J Surg Pathol | year= 1979 | volume= 3 | issue= 6 | pages= 507-23 | pmid=534388 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=534388  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid192432&amp;quot;&amp;gt;{{cite journal| author=Dahlin DC, Unni KK, Matsuno T| title=Malignant (fibrous) histiocytoma of bone--fact or fancy?. | journal=Cancer | year= 1977 | volume= 39 | issue= 4 | pages= 1508-16 | pmid=192432 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=192432  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Dedifferentiated liposarcoma represents both a morphologically and biologically fascinating lesion in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed. First described in 1979 by Evans,22 who coopted the term from Dhalin et al’s23 description of tumor progression in chondrosarcoma, dedifferentiation in well- differentiated liposarcoma tends to occur more fre- quently in the primary tumor (90%) but can also be observed in recurrences (10%). The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024144</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024144"/>
		<updated>2014-09-19T19:54:17Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Dedifferentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
In this form of liposarcoma there is a transition from a low-grade differentiation, to a high-grade differentiation within the same mass of well-differentiated liposarcoma. &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Dedifferentiated liposarcoma represents both a morphologically and biologically fascinating lesion in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed. First described in 1979 by Evans,22 who coopted the term from Dhalin et al’s23 description of tumor progression in chondrosarcoma, dedifferentiation in well- differentiated liposarcoma tends to occur more fre- quently in the primary tumor (90%) but can also be observed in recurrences (10%). The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
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==Associated Conditions==&lt;br /&gt;
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==Gross Pathology==&lt;br /&gt;
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==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024136</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024136"/>
		<updated>2014-09-19T19:39:15Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
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&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Dedifferentiated liposarcoma represents both a mor- phologically and biologically fascinating lesion in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed. First described in 1979 by Evans,22 who coopted the term from Dhalin et al’s23 description of tumor progression in chondrosarcoma, dedifferentiation in well- differentiated liposarcoma tends to occur more fre- quently in the primary tumor (90%) but can also be observed in recurrences (10%). The transition usually occurs in an abrupt fashion (Fig 8); however, in some cases this can be more gradual and exceptionally low- grade and high-grade areas appear to be intermingled. Dedifferentiated areas exhibit a variable histologic pic- ture but most frequently it overlaps with storiform and pleomorphic malignant fibrous histiocytoma (Fig 9) as well as myxofibrosarcoma. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low-grade and high-grade sub- types.18,24 Low-grade dedifferentiation was defined by the presence of bland spindle cells organized in a fascicular pattern and exhibiting a cellularity intermedi- ate between well-differentiated sclerosing liposarcoma and usual high-grade areas. The very existence of low-grade dedifferentiation raises the question of the differential diagnosis with spindle cell liposarcoma. However, it should be noted that spindle cell liposar- coma represents a lipogenic lesion (eg, it contains atypical adipocytes or lipoblasts) whereas dedifferenti- ated areas, both low- and high-grade, are generally nonlipogenic.&lt;br /&gt;
&lt;br /&gt;
Dedifferentiated liposarcoma may exhibit heterolo- gous differentiation in approximately 5% to 10% of cases, which apparently does not affect the clinical outcome. Most often the line of heterologous differentia- tion is myogenic (Figs 10-12) or osteo/chondrosarcoma- tous, but angiosarcomatous elements also have been reported. Recently, a peculiar ‘‘neural-like’’ or ‘‘meningo- thelial-like whorling pattern of dedifferentiation has been described but both immunohistochemistry and electron microscopy failed to elucidate the line of differ- entiation in these lesions25,26 (Fig 13). This pattern is often associated with ossification (Fig 14).  Surprisingly, the biological behavior of dedifferenti- ated liposarcoma tends to be less aggressive than that of other types of high-grade pleomorphic sarcomas.17,18 At variance with well-differentiated liposarcoma, dediffer- entiation is associated with a 15% to 20% metastatic rate. However, long-term survival rates for retroperito- neal dedifferentiated liposarcoma is not significantly worse than that observed in ordinary well-differentiated liposarcoma occurring at the same site, mortality being more related to repeated destructive local recurrences than to metastatic spread. The fact that dedifferenti- ated liposarcoma can recur as an entirely well-differenti- ated liposarcoma also represents a morphologic indica- tor of the unique biologic behavior of this liposarcoma subtype.&lt;br /&gt;
&lt;br /&gt;
Genetics as well as molecular pathology have recently provided data that, at least in part, may account for the discrepancy observed between morphology and biologic aggressiveness. Interestingly, in stark contrast with the complex karyotypic aberrations observed in pleomor- phic sarcomas, dedifferentiated liposarcoma usually retains the same basic cytogenetic anomalies as well- differentiated liposarcoma represented by the presence of ring and/or giant marker chromosomes,27 although there may be superimposed additional aberrations.28 A significant increase in the level of both MDM2 overex- pression and amplification in the high-grade areas has been observed that may account for the tumor progres- sion in this subset of sarcomas.29,30 However, at variance with high-grade pleomorphic sarcomas, in which con- comitant MDM2 amplification and TP53 mutation are detected31 and that are significantly related to poor clinical outcome, retention of TP53 integrity is almost always observed in dedifferentiated liposarcoma.29 There- fore, dedifferentiated liposarcoma appears to be just one more step along the spectrum of well-differentiated adipocytic neoplasms, showing only an increased dosage of the same molecular targets involved in the molecular pathogenesis of well-differentiated liposarcoma.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
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==Associated Conditions==&lt;br /&gt;
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==Gross Pathology==&lt;br /&gt;
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==Microscopic Pathology==&lt;br /&gt;
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==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024133</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024133"/>
		<updated>2014-09-19T19:35:19Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
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&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged: atypical lipoma and atypical lipomatous tumor. However, there is still no consensus on when to use each term.&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024128</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024128"/>
		<updated>2014-09-19T19:25:26Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of &#039;&#039;&#039;recurrence&#039;&#039;&#039; and &#039;&#039;&#039;no potential to metastasize&#039;&#039;&#039;, unless dedifferentiation occurs, two terms have emerged:&amp;lt;ref name=&amp;quot;pmid421182&amp;quot;&amp;gt;{{cite journal| author=Evans HL, Soule EH, Winkelmann RK| title=Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. | journal=Cancer | year= 1979 | volume= 43 | issue= 2 | pages= 574-84 | pmid=421182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=421182  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Atypical lipoma - &lt;br /&gt;
&lt;br /&gt;
*Atypical lipomatous tumor - &lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024126</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024126"/>
		<updated>2014-09-19T19:23:53Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Because well-differentiated lipomas are characterized by having approximately 30% risk of recurrence and no potential to metastasize, unless dedifferentiation occurs, two terms have emerged:&lt;br /&gt;
*Atypical lipoma - &lt;br /&gt;
&lt;br /&gt;
*Atypical lipomatous tumor - &lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024104</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024104"/>
		<updated>2014-09-19T19:06:29Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Genetics */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the amount of rearranged gene present in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024098</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024098"/>
		<updated>2014-09-19T19:01:42Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Genetics */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogene]]s, including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;  Because this same region is rearranged in benign lipomas as well, this last may integrate a well-defferentiated liposarcoma mass.  The difference between these malignant and benign tissues resides in the effect of the amount of rearranged gene in each one.&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt; &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
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==Associated Conditions==&lt;br /&gt;
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==Gross Pathology==&lt;br /&gt;
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==Microscopic Pathology==&lt;br /&gt;
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==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
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{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Protoonogenes&amp;diff=1024081</id>
		<title>Protoonogenes</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Protoonogenes&amp;diff=1024081"/>
		<updated>2014-09-19T18:55:32Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: ←Redirected page to Proto-oncogene&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;#REDIRECT:[[Proto-oncogene]]&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Protooncogene&amp;diff=1024079</id>
		<title>Protooncogene</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Protooncogene&amp;diff=1024079"/>
		<updated>2014-09-19T18:54:11Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: ←Redirected page to Proto-oncogene&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;#REDIRECT:[[Proto-oncogene]]&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Proto-oncogene&amp;diff=1024078</id>
		<title>Proto-oncogene</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Proto-oncogene&amp;diff=1024078"/>
		<updated>2014-09-19T18:53:25Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{CMG}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
A &#039;&#039;&#039;proto-oncogene&#039;&#039;&#039; is a normal gene that can become an oncogene due to mutations or increased [[Gene expression|expression]]. The resultant protein may be termed as &#039;&#039;&#039;oncoprotein&#039;&#039;&#039;.&amp;lt;ref name=Kumar20&amp;gt;Chapter 20 - NEOPLASMS OF THE THYROID - in: {{cite book |author=Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson |title=Robbins Basic Pathology|publisher=Saunders |location=Philadelphia |pages= |isbn=1-4160-2973-7 |oclc= |doi=}} 8th edition.&amp;lt;/ref&amp;gt; Proto-oncogenes code for [[protein]]s that help to regulate [[cell growth]] and [[cell differentiation|differentiation]]. Proto-oncogenes are often involved in [[signal transduction]] and execution of [[mitosis|mitogenic]] signals, usually through their [[protein]] products. Upon &#039;&#039;activation&#039;&#039;, a proto-oncogene (or its product) becomes a tumor-inducing agent, an oncogene.&amp;lt;ref&amp;gt;{{cite journal |author=Todd R, Wong DT |title=Oncogenes |journal=Anticancer Res. |volume=19 |issue=6A |pages=4729–46 |year=1999 |pmid=10697588}}&amp;lt;/ref&amp;gt; Examples of proto-oncogenes include [[Ras (protein)|RAS]], [[Wnt signaling pathway|WNT]], [[Myc|MYC]], [[Extracellular signal-regulated kinases|ERK]], and [[Trk receptor|TRK]]. The MYC gene is implicated in Burkitt&#039;s Lymphoma, which starts when a chromosomal translocation moves an enhancer sequence within the vicinity of the MYC gene. The MYC gene codes for widely used transcription factors. When the enhancer sequence is wrongly placed, these transcription factors are produced at much higher rates. Another example of an oncogene is the [[Bcr-Abl]] gene found on the Philadelphia Chromosome, a piece of genetic material seen in Chronic Myelogenous Leukemia caused by the translocation of pieces from chromosomes 9 and 22. Bcr-Abl codes for a receptor tyrosine kinase, which is constitutively active, leading to uncontrolled cell proliferation. (More information about the Philadelphia Chromosome below)&lt;br /&gt;
&lt;br /&gt;
==Proto-Oncogene==&lt;br /&gt;
=== Activation ===&lt;br /&gt;
The proto-oncogene can become an oncogene by a relatively small modification of its original function. There are three basic methods of activation:&lt;br /&gt;
#A [[mutation]] within a proto-oncogene, or within a regulatory region (for example the promoter region), can cause a change in the protein structure, causing&lt;br /&gt;
#* an increase in protein ([[enzyme]]) activity&lt;br /&gt;
#* a loss of [[Regulation of gene expression|regulation]]&lt;br /&gt;
# An increase in the amount of a certain protein (protein concentration), caused by&lt;br /&gt;
#* an increase of protein expression (through misregulation)&lt;br /&gt;
#* an increase of protein (mRNA) stability, prolonging its existence and thus its activity in the cell&lt;br /&gt;
#* [[gene duplication]] (one type of [[Chromosome abnormalities|chromosome abnormality]]), resulting in an increased amount of protein in the cell&lt;br /&gt;
# A [[chromosomal translocation]] (another type of [[Chromosome abnormalities|chromosome abnormality]])&lt;br /&gt;
#*There are 2 different types of chromosomal translocations that can occur:&lt;br /&gt;
##translocation events which relocate a proto-oncogene to a new chromosomal site that leads to higher expression&lt;br /&gt;
##translocation events that lead to a fusion between a proto-oncogene and a 2nd gene (this creates a fusion protein with increased cancerous/oncogenic activity)&lt;br /&gt;
##* the expression of a constitutively active &#039;&#039;hybrid protein&#039;&#039;. This type of mutation in a dividing [[stem cell]] in the [[bone marrow]] leads to adult [[leukemia]]&lt;br /&gt;
##*Philadelphia Chromosome is an example of this type of translocation event.  This chromosome was discovered in 1960 by [[Peter Nowell]] and David Hungerford, and it is a fusion of parts of DNA from chromosome 22 and chromosome 9. The broken end of chromosome 22 contains the &amp;quot;BCR&amp;quot; gene, which fuses with a fragment of chromosome 9 that contains the &amp;quot;ABL1&amp;quot; gene.  When these two chromosome fragments fuse the genes also fuse creating a new gene: &amp;quot;BCR-ABL&amp;quot;.  This fused gene encodes for a protein that displays high protein tyrosine kinase activity (this activity is due to the &amp;quot;ABL1&amp;quot; half of the protein). The unregulated expression of this protein activates other proteins that are involved in cell cycle and cell division which can cause a cell to grow and divide uncontrollably (the cell becomes cancerous).  As a result, the Philadelphia Chromosome is associated with Chronic Myelogenous Leukemia (as mentioned before) as well as other forms of Leukemia.&amp;lt;ref&amp;gt;{{cite journal|last=Chial|first=H|title=Proto-oncogenes to Oncogenes to Cancer|journal=Nature Education|year=2008|volume=1|issue=1}}&amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
The expression of oncogenes can be regulated by [[microRNA]]s (miRNAs), small [[RNA]]s 21-25 nucleotides in length that control gene expression by [[downregulate|downregulating]] them.&amp;lt;ref name=Negrini&amp;gt;{{cite journal |author=Negrini M, Ferracin M, Sabbioni S, Croce CM |title=MicroRNAs in human cancer: from research to therapy |journal=J Cell Sci. |volume=120 |issue=Pt 11 |pages=1833–40 |date=Jun 2007 |pmid=17515481 |doi=10.1242/jcs.03450 }}&amp;lt;/ref&amp;gt; Mutations in such [[microRNAs]] (known as [[oncomir]]s) can lead to activation of oncogenes.&amp;lt;ref name=&amp;quot;Esquela-Kerscher&amp;quot;&amp;gt;{{cite journal |author=Esquela-Kerscher A, Slack FJ |title=Oncomirs - microRNAs with a role in cancer |journal=Nat Rev Cancer |volume=6 |issue=4 |pages=259–69 |date=Apr 2006 |pmid=16557279 |doi=10.1038/nrc1840 }}&amp;lt;/ref&amp;gt; [[Antisense]] messenger RNAs could theoretically be used to block the effects of oncogenes.&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024073</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024073"/>
		<updated>2014-09-19T18:48:20Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Genetics */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt;  This chromosome region is rich in [[protooncogenes]], including the CHOP, CDK4, MDM2, HMGI-C, GLI, SAS, OS1, and the OS9, which play an important role in the [[pathogenesis]] of many [[neoplasms]].  Amplification of some of these regions, with concomitant amplification of their proteins has been demonstrated in this type of [[soft tissue sarcoma]].&amp;lt;ref name=&amp;quot;pmid10727978&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Doglioni C, Piccinin S, Sciot R, Furlanetto A, Boiocchi M et al.| title=Coordinated expression and amplification of the MDM2, CDK4, and HMGI-C genes in atypical lipomatous tumours. | journal=J Pathol | year= 2000 | volume= 190 | issue= 5 | pages= 531-6 | pmid=10727978 | doi=10.1002/(SICI)1096-9896(200004)190:5&amp;lt;531::AID-PATH579&amp;gt;3.0.CO;2-W | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10727978  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024068</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024068"/>
		<updated>2014-09-19T18:41:53Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Genetics */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
===Well-Differentiated Liposarcoma===&lt;br /&gt;
Specific diagnostic techniques, such as karyotipic analysis, have showed a specific characteristic of these cells, namely the presence of a large marker chromosome and/or of an extra ring.&amp;lt;ref name=&amp;quot;pmid8827023&amp;quot;&amp;gt;{{cite journal| author=Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N et al.| title=Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group). | journal=Am J Surg Pathol | year= 1996 | volume= 20 | issue= 10 | pages= 1182-9 | pmid=8827023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8827023  }} &amp;lt;/ref&amp;gt;  With the help of in situ hybridization, these rings and/or large markers were noted to accommodate amplified genetic sequences of the 12q13-15 chromosome region.&amp;lt;ref name=&amp;quot;pmid8353809&amp;quot;&amp;gt;{{cite journal| author=Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W et al.| title=Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors. | journal=Cancer Genet Cytogenet | year= 1993 | volume= 68 | issue= 2 | pages= 85-90 | pmid=8353809 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8353809  }} &amp;lt;/ref&amp;gt; &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024050</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024050"/>
		<updated>2014-09-19T18:26:32Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&#039;&#039;&#039;Spindle cell lipocarcinoma&#039;&#039;&#039; is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024044</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024044"/>
		<updated>2014-09-19T18:22:01Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Spindle cell lipocarcinoma is a rare adult type of well-differentiated liposarcoma.  It results from the proliferation of neural-like spindle cells, organized in a fibrous structure, containing lipoblasts.&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
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[[Category:Types of cancer]]&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_differential_diagnosis&amp;diff=1024040</id>
		<title>Liposarcoma differential diagnosis</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_differential_diagnosis&amp;diff=1024040"/>
		<updated>2014-09-19T18:17:01Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
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{{Liposarcoma}}&lt;br /&gt;
&lt;br /&gt;
Please help WikiDoc by adding more content here.  It&#039;s easy!  Click  [[Help:How_to_Edit_a_Page|here]]  to learn about editing.&lt;br /&gt;
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&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
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	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024039</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024039"/>
		<updated>2014-09-19T18:14:15Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
Spindle cell lipocarcinoma, a rare adult type of well-differentiated liposarcoma&amp;lt;ref name=&amp;quot;pmid8067512&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP, Mentzel T, Newman PL, Fletcher CD| title=Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal=Am J Surg Pathol | year= 1994 | volume= 18 | issue= 9 | pages= 913-21 | pmid=8067512 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=8067512  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid5&amp;quot;&amp;gt;{{cite journal| author=Hendrickson WA, Ward KB| title=Atomic models for the polypeptide backbones of myohemerythrin and hemerythrin. | journal=Biochem Biophys Res Commun | year= 1975 | volume= 66 | issue= 4 | pages= 1349-56 | pmid=5 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=5  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024026</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1024026"/>
		<updated>2014-09-19T17:59:29Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;  For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposarcoma was described under the term spindle cell liposarcoma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023989</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023989"/>
		<updated>2014-09-19T16:30:27Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
For the diagnosis of inflammatory liposarcoma a large sample is required to avoid missing the adipocitic component of the neoplasm.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_differential_diagnosis&amp;diff=1023987</id>
		<title>Liposarcoma differential diagnosis</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_differential_diagnosis&amp;diff=1023987"/>
		<updated>2014-09-19T16:27:44Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
&lt;br /&gt;
Please help WikiDoc by adding more content here.  It&#039;s easy!  Click  [[Help:How_to_Edit_a_Page|here]]  to learn about editing.&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Needs content]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
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[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
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{{WikiDoc Help Menu}}&lt;br /&gt;
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		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023986</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023986"/>
		<updated>2014-09-19T16:23:21Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.  The inflammatory component is frequently composed of different lympho-plasmacytic aggregates.  These tend to be predominantly formed by a specific type of B-cell yet, T-cells may in some cases populate the inflammatory aggregate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023983</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023983"/>
		<updated>2014-09-19T16:16:03Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9158675&amp;quot;&amp;gt;{{cite journal| author=Kraus MD, Guillou L, Fletcher CD| title=Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 5 | pages= 518-27 | pmid=9158675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9158675  }} &amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid9255251&amp;quot;&amp;gt;{{cite journal| author=Argani P, Facchetti F, Inghirami G, Rosai J| title=Lymphocyte-rich well-differentiated liposarcoma: report of nine cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 8 | pages= 884-95 | pmid=9255251 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=9255251  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023981</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023981"/>
		<updated>2014-09-19T16:12:13Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
Its adipocitic nature may be misidentified due to the heavy chronic inflammatory infiltrate.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023980</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023980"/>
		<updated>2014-09-19T16:05:53Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, since multiple benign lesions may contain lipoblasts; nor are they identified in every [[liposarcoma]], as its absence does not prevent the diagnosis of the condition, if remaining criteria are met.  Lipoblasts may &amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023973</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023973"/>
		<updated>2014-09-19T15:57:18Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
In general, adipocytic [[neoplasms]] are often identified by the presence of these lipoblasts, however, its presence is not synonym, nor are they identified in every liposarcoma.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023970</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023970"/>
		<updated>2014-09-19T15:50:43Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]].  Besides these two types of cells, mono or multivacuolated lipoblasts may also be identified.  These last are characterized by the presence of single (mono) or multiple (multi) peripheral cytoplasmic vacuoles that press on the hyperchromatic nucleus.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
In general, [[adipocytic]] [[neoplasms]] are often identified by the presence of these lipoblasts.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023960</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023960"/>
		<updated>2014-09-19T15:37:17Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
Frequently composed by [[adipocytes]] with different cell sizes, hyperchromasia and nuclear atypia.  [[Fibrous]] septa may be identified among [[adipocytes]], containing hyperchromatic [[stromal cells]]. &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023942</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023942"/>
		<updated>2014-09-19T15:26:09Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions.  The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.  In certain cases the fibrous component of the [[neoplasm]] may occupy most of its mass.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
&lt;br /&gt;
Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023933</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023933"/>
		<updated>2014-09-19T15:21:27Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions. &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
===Sclerosing Liposarcoma===&lt;br /&gt;
The particular [[histological]] finding in this type of well differentiated liposarcoma is the identification of distinctive [[stromal]] cells distributed across the tissue, and associated with lipoblasts filled with multiple [[vacuoles]].  This association forms a [[collagenous]] background of fibrillary appearance.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023914</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023914"/>
		<updated>2014-09-19T15:14:32Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
Occurs most frequently at the retroperitoneum and paratesticular regions. &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Microscopically, the main histologic finding is the presence of scattered, distinctive, bizarre stromal cells associated with rare multivacuolated lipoblasts set in a fibrillary collagenous background (Fig 3). Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023908</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023908"/>
		<updated>2014-09-19T15:10:31Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
According to the WHO classification described previously, well differentiated liposarcomas may be sub-classified into 3 types: &#039;&#039;sclerosing&#039;&#039;; &#039;&#039;adipocytic&#039;&#039;; and &#039;&#039;inflammatory&#039;&#039;.&lt;br /&gt;
====Sclerosing Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
====Adipocytic Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
====Inflammatory Liposarcoma====&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Sclerosing liposarcoma is characterized clinically by the tendency to occur most frequently in the retroperito- neum and in the paratesticular region. Microscopically, the main histologic finding is the presence of scattered, distinctive, bizarre stromal cells associated with rare multivacuolated lipoblasts set in a fibrillary collagenous background (Fig 3). Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023900</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023900"/>
		<updated>2014-09-19T15:05:30Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the [[limbs]] and [[retroperitoneum]] in equal frequency, and occasionally at the [[mediastinum]] and [[spermatic cord]], representing about 45% of liposarcomas.&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Morphologi- cally, the WHO classification recognizes three subtypes of well-differentiated liposarcoma: adipocytic (or lipoma- like), sclerosing, and inflammatory. Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Sclerosing liposarcoma is characterized clinically by the tendency to occur most frequently in the retroperito- neum and in the paratesticular region. Microscopically, the main histologic finding is the presence of scattered, distinctive, bizarre stromal cells associated with rare multivacuolated lipoblasts set in a fibrillary collagenous background (Fig 3). Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023896</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023896"/>
		<updated>2014-09-19T15:01:57Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Pathogenesis */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
This type of liposarcoma occurs both at the limbs and retroperitoneum, representing about 45% of liposarcomas.&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
 Spermatic cord and mediastinum represent less-fre- quent but well-known anatomic locations. Morphologi- cally, the WHO classification recognizes three subtypes of well-differentiated liposarcoma: adipocytic (or lipoma- like), sclerosing, and inflammatory. Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Sclerosing liposarcoma is characterized clinically by the tendency to occur most frequently in the retroperito- neum and in the paratesticular region. Microscopically, the main histologic finding is the presence of scattered, distinctive, bizarre stromal cells associated with rare multivacuolated lipoblasts set in a fibrillary collagenous background (Fig 3). Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023892</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023892"/>
		<updated>2014-09-19T14:55:27Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Well Differentiated Liposarcoma */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
Well-differentiated liposarcoma represents approxi- mately 40% to 45% of all liposarcomas and tends to occur equally in the retroperitoneum or the limbs. Spermatic cord and mediastinum represent less-fre- quent but well-known anatomic locations. Morphologi- cally, the WHO classification recognizes three subtypes of well-differentiated liposarcoma: adipocytic (or lipoma- like), sclerosing, and inflammatory. Adipocytic liposar- coma is mostly composed of mature adipocytes exhibit- ing striking variation in cell size and at least focal nuclear atypia and hyperchromasia (Fig 1). Scattered hyperchromatic stromal cells may be encountered within fibrous septa. Of course, a varying number (from many to none) of monovacuolated or multivacuolated lipo- blasts (defined by the presence of single or multiple sharply marginated cytoplasmic vacuoles that indent an enlarged hyperchromatic nucleus) may be found (Fig 2). It is a commonly held opinion that lipoblasts represent the hallmark for any type of malignant adipocytic lesion. &lt;br /&gt;
&lt;br /&gt;
However, it is important to emphasize that the mere presence of lipoblasts does not make (or is required for) a diagnosis of liposarcoma. As a matter of fact, there exist a number of entirely benign adipocytic lesions (eg, lipoblastoma, pleomorphic lipoma, chondroid lipoma) that may contain numerous lipoblasts. By contrast, the absence of lipoblasts within an adipocytic neoplasm fulfilling all the remaining diagnostic criteria for liposar- coma does not prevent such a diagnosis.&lt;br /&gt;
&lt;br /&gt;
Sclerosing liposarcoma is characterized clinically by the tendency to occur most frequently in the retroperito- neum and in the paratesticular region. Microscopically, the main histologic finding is the presence of scattered, distinctive, bizarre stromal cells associated with rare multivacuolated lipoblasts set in a fibrillary collagenous background (Fig 3). Occasionally the fibrous component represents the vast majority of the neoplasm to the extent that lipogenic areas can be easily overlooked or even missed in a small tissue sample (Fig 4).&lt;br /&gt;
&lt;br /&gt;
Another morphologic variation that represents a potential diagnostic pitfall is the presence of a chronic inflammatory infiltrate to the extent that the adipocytic nature of the neoplasm can be obscured (Fig 5). The existence of examples of liposarcoma characterized by the presence of prominent mononuclear inflammatory infiltrates has been acknowledged since the publication of Stout’s5 classification of liposarcoma. Nonetheless, reports dealing specifically with this subtype have not been available until very recently.6,7 The inflammatory infiltrate is usually composed of polyphenotypic lympho- plasmacytic aggregates in which a B-cell phenotype tends to predominate. However, cases exist in which a T-cell population represents the main inflammatory component. Differential diagnosis includes nonadipo- cytic lesions such as inflammatory myofibroblastic tu- mor and Castleman’s disease. Careful as well as exten- sive sampling is mandatory to permit recognition of the adipocytic component that otherwise could be easily missed. The presence of bizarre multinucleate stromal cells represents a useful diagnostic clue.&lt;br /&gt;
&lt;br /&gt;
In 1994, a rare variant of well-differentiated liposar- coma was described under the term spindle cell liposar- coma,8,9 which occurs in adults and, at least in the first series, appeared to relatively often involve subcutaneous soft tissue. However, through observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Morpho- logically, spindle cell liposarcoma is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background (Fig 6) and is associ- ated with an atypical lipomatous component that usu- ally includes lipoblasts (Fig 7). Main differential diag- noses include spindle cell lipoma (composed of bland, sometimes palisading, CD34-positive spindle cells, ad- mixed with eosinophilic refractile collagen bundles), neurofibroma (characterized by a less cellular S-100– positive spindle cell proliferation with wavy nuclei), dermatofibrosarcoma protuberans (cytologically bland, monomorphic CD34-positive spindle cell proliferation organized in a distinctive storiform growth pattern and characterized by tendency to infiltrate the surrounding fat in a peculiar ‘‘honeycomb’’ pattern), malignant peripheral nerve sheath tumor (generally highly cellular tumors composed of tapering or wavy spindle cells featuring perivascular accentuation and focal S-100– positive immunoreactivity in approximately 50% of cases), and well-differentiated sclerosing liposarcoma (characterized by the presence of bizarre hyperchro- matic stromal cells set in fibrillary collagen). The pres- ence of an atypical lipomatous component also permits distinction from low-grade fibromyxoid sarcoma (Evans’ tumor). Interestingly, spindle cell liposarcoma exhibits chromosome changes (ring chromosomes and giant marker chromosomes) identical to those observed in other members of the well-differentiated liposarcoma group, thus validating its classification.&lt;br /&gt;
&lt;br /&gt;
As previously mentioned, the integration between morphologic and cytogenetic observations has proved extremely useful, and adipocytic neoplasms represent one of the fields of surgical pathology wherein such a synergy has been most fruitful (Table 2). Karyotypic analysis of well-differentiated liposarcoma has shown that this group of mesenchymal neoplasms is marked by the presence of extra ring and/or giant marker chromo- somes.10 Fluorescence in situ hybridization studies have demonstrated that these rings and giant markers con- tain amplified sequences derived from the 12q13-15 chromosome region.11 The 12q13-15 chromosome re- gion is very complex and contains several important protooncogenes, such as MDM2, CDK4, HMGI-C, SAS, GLI, CHOP, OS1, and OS9, known to play an important role in the molecular pathogenesis of many neoplastic processes. Interestingly, concomitant amplification of HMGI-C (a gene encoding for an architectural transcrip- tion factor involved in adipocytic differentiation), MDM2, and CDK4 (both acting as positive regulators of cell cycle progression at the G1-S checkpoint), as well as overexpression of the proteins thereof has been recently demonstrated in well-differentiated liposarcomas.12 The fact that the 12q13-15 region is also rearranged in ordinary benign lipomas (leading to HMGI-C activa- tion) and the observation of 12q15 duplication in adipo- cytic neoplasms exhibiting minimal atypical changes (C.D.M. Fletcher, unpublished observation) have led to the postulation that ordinary lipomas may form a morphologic and genetic continuum with well-differenti- ated liposarcoma, with the degree of atypia being a gene dosage effect.12&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
 &lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023889</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023889"/>
		<updated>2014-09-19T14:53:51Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
According to their class, each liposarcoma will have specific characteristics and pathogenesis:&lt;br /&gt;
===Well Differentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Dedifferentiated Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Myxoid Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Round Cell Liposarcoma===&lt;br /&gt;
&lt;br /&gt;
===Pleomorphic Liposarcoma===&lt;br /&gt;
 &lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023887</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023887"/>
		<updated>2014-09-19T14:50:45Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
&lt;br /&gt;
==Genetics==&lt;br /&gt;
&lt;br /&gt;
==Associated Conditions==&lt;br /&gt;
&lt;br /&gt;
==Gross Pathology==&lt;br /&gt;
&lt;br /&gt;
==Microscopic Pathology==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023885</id>
		<title>Liposarcoma pathophysiology</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_pathophysiology&amp;diff=1023885"/>
		<updated>2014-09-19T14:49:40Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}} {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Pathogenesis==&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023883</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023883"/>
		<updated>2014-09-19T14:48:42Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
Liposarcoma, a type of soft tissue sarcoma may be classified into: well-differentiated, dedifferentiated, myxoid, round cell, or pleiomorphic liposarcoma.&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
{| style=&amp;quot;border: 0px; font-size: 90%; margin: 3px;&amp;quot; align=center&lt;br /&gt;
|+&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 500px;&amp;quot; | {{fontcolor|#FFF|WHO Classification of Liposarcoma}}&lt;br /&gt;
|-&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #F5F5F5;&amp;quot; |&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
|-&lt;br /&gt;
|}&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023881</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023881"/>
		<updated>2014-09-19T14:45:17Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
{| style=&amp;quot;border: 0px; font-size: 90%; margin: 3px;&amp;quot; align=center&lt;br /&gt;
|+&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 500px;&amp;quot; | {{fontcolor|#FFF|WHO Classification of Liposarcoma}}&lt;br /&gt;
|-&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #F5F5F5;&amp;quot; |&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
|-&lt;br /&gt;
|}&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023874</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023874"/>
		<updated>2014-09-19T14:39:46Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
&lt;br /&gt;
{| style=&amp;quot;border: 0px; font-size: 90%; margin: 3px;&amp;quot; align=center&lt;br /&gt;
|+&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 500px;&amp;quot; | {{fontcolor|#FFF|WHO Classification of Liposarcoma}}&lt;br /&gt;
|-&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #F5F5F5;&amp;quot; |&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
|-&lt;br /&gt;
|}&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
it is useful to think of liposarcomas as three large groups from a conceptual point of view. ALN/WDL, also termed atypical lipomatous neoplasm (ALN) when it occurs in superficial soft tissue or in the muscles of the extremity because of its low-grade behavior, and dedifferentiated liposarcoma (DL) comprise one subgroup. Widely disparate in terms of biologic behavior, they are closely related from a pathogenetic point of view because a subset of ALN/WDL histologically progresses to dedifferentiated sarcomas. With dedifferentiation, the tumor acquires metastatic potential, a phenomenon accompanied by additional cytogenetic abnormalities. The second group is myxoid liposarcoma that ranges in appearance from pure myxoid tumors at one extreme to poorly differentiated round cell (poorly differentiated myxoid) tumors at the other. Pleomorphic liposarcomas are rare, poorly characterized tumors, many of which resemble undifferentiated pleomorphic sarcoma, except for the presence of pleomorphic lipoblasts. Finally, a small number of liposarcomas exhibit unusual features or combine patterns not accounted for in the previous classification (liposarcomas of mixed type). These are best individualized and diagnosed as liposarcomas of mixed or unclassifiable type, recognizing that the number of such lesions is dwindling because of the ability of molecular testing to assign them to a category.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023871</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023871"/>
		<updated>2014-09-19T14:38:44Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{| style=&amp;quot;border: 0px; font-size: 90%; margin: 3px;&amp;quot; align=center&lt;br /&gt;
|+&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 500px;&amp;quot; | {{fontcolor|#FFF|Significance}}&lt;br /&gt;
|-&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #F5F5F5;&amp;quot; |&lt;br /&gt;
|-&lt;br /&gt;
|}&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
it is useful to think of liposarcomas as three large groups from a conceptual point of view. ALN/WDL, also termed atypical lipomatous neoplasm (ALN) when it occurs in superficial soft tissue or in the muscles of the extremity because of its low-grade behavior, and dedifferentiated liposarcoma (DL) comprise one subgroup. Widely disparate in terms of biologic behavior, they are closely related from a pathogenetic point of view because a subset of ALN/WDL histologically progresses to dedifferentiated sarcomas. With dedifferentiation, the tumor acquires metastatic potential, a phenomenon accompanied by additional cytogenetic abnormalities. The second group is myxoid liposarcoma that ranges in appearance from pure myxoid tumors at one extreme to poorly differentiated round cell (poorly differentiated myxoid) tumors at the other. Pleomorphic liposarcomas are rare, poorly characterized tumors, many of which resemble undifferentiated pleomorphic sarcoma, except for the presence of pleomorphic lipoblasts. Finally, a small number of liposarcomas exhibit unusual features or combine patterns not accounted for in the previous classification (liposarcomas of mixed type). These are best individualized and diagnosed as liposarcomas of mixed or unclassifiable type, recognizing that the number of such lesions is dwindling because of the ability of molecular testing to assign them to a category.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023870</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023870"/>
		<updated>2014-09-19T14:38:17Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
{| style=&amp;quot;border: 0px; font-size: 90%; margin: 3px;&amp;quot; align=center&lt;br /&gt;
|+&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 100px;&amp;quot; | {{fontcolor|#FFF|Diagnostic Markers}}&lt;br /&gt;
! style=&amp;quot;background: #4479BA; width: 500px;&amp;quot; | {{fontcolor|#FFF|Significance}}&lt;br /&gt;
|-&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #DCDCDC;&amp;quot; | &#039;&#039;&#039;&#039;&#039;&#039;&lt;br /&gt;
| style=&amp;quot;padding: 5px 5px; background: #F5F5F5;&amp;quot; |&lt;br /&gt;
*Positive in persons exposed to HDV&lt;br /&gt;
*Persists, even after viral clearance&lt;br /&gt;
|-&lt;br /&gt;
|}&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
&amp;lt;!--&lt;br /&gt;
it is useful to think of liposarcomas as three large groups from a conceptual point of view. ALN/WDL, also termed atypical lipomatous neoplasm (ALN) when it occurs in superficial soft tissue or in the muscles of the extremity because of its low-grade behavior, and dedifferentiated liposarcoma (DL) comprise one subgroup. Widely disparate in terms of biologic behavior, they are closely related from a pathogenetic point of view because a subset of ALN/WDL histologically progresses to dedifferentiated sarcomas. With dedifferentiation, the tumor acquires metastatic potential, a phenomenon accompanied by additional cytogenetic abnormalities. The second group is myxoid liposarcoma that ranges in appearance from pure myxoid tumors at one extreme to poorly differentiated round cell (poorly differentiated myxoid) tumors at the other. Pleomorphic liposarcomas are rare, poorly characterized tumors, many of which resemble undifferentiated pleomorphic sarcoma, except for the presence of pleomorphic lipoblasts. Finally, a small number of liposarcomas exhibit unusual features or combine patterns not accounted for in the previous classification (liposarcomas of mixed type). These are best individualized and diagnosed as liposarcomas of mixed or unclassifiable type, recognizing that the number of such lesions is dwindling because of the ability of molecular testing to assign them to a category.&lt;br /&gt;
&lt;br /&gt;
--&amp;gt;&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023866</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023866"/>
		<updated>2014-09-19T14:30:40Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023865</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023865"/>
		<updated>2014-09-19T14:30:07Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: /* Classification */&lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  &lt;br /&gt;
&lt;br /&gt;
&lt;br /&gt;
histologically, biologically, cytogenetically, and, by molecular analyses&lt;br /&gt;
&lt;br /&gt;
Liposarcomas may be classified into different types, according to their histologic, cytogenetic, biologic and molecular features.  The [[WHO]] has proposed the following classification of liposarcomas:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
	<entry>
		<id>https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023863</id>
		<title>Liposarcoma classification</title>
		<link rel="alternate" type="text/html" href="https://www.wikidoc.org/index.php?title=Liposarcoma_classification&amp;diff=1023863"/>
		<updated>2014-09-19T14:26:52Z</updated>

		<summary type="html">&lt;p&gt;Joao Silva: &lt;/p&gt;
&lt;hr /&gt;
&lt;div&gt;__NOTOC__&lt;br /&gt;
{{Liposarcoma}}&lt;br /&gt;
{{CMG}}; {{AE}} {{JS}}&lt;br /&gt;
&lt;br /&gt;
==Overview==&lt;br /&gt;
&lt;br /&gt;
==Classification==&lt;br /&gt;
Liposarcoma is one type of [[soft tissue sarcoma]]s, which accounts for about 20% of the [[mesenchymal]] [[malignancies]].  According to the [[WHO]], liposarcoma may be classified as:&amp;lt;ref&amp;gt;{{cite book | last = Weiss | first = Sharon | title = Histological typing of soft tissue tumours | publisher = Springer-Verlag | location = Berlin New York | year = 1994 | isbn = 3540567941 }}&amp;lt;/ref&amp;gt;&amp;lt;ref name=&amp;quot;pmid10982304&amp;quot;&amp;gt;{{cite journal| author=Dei Tos AP| title=Liposarcoma: new entities and evolving concepts. | journal=Ann Diagn Pathol | year= 2000 | volume= 4 | issue= 4 | pages= 252-66 | pmid=10982304 | doi=10.1053/adpa.2000.8133 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&amp;amp;tool=sumsearch.org/cite&amp;amp;retmode=ref&amp;amp;cmd=prlinks&amp;amp;id=10982304  }} &amp;lt;/ref&amp;gt;&lt;br /&gt;
*Well-diferentiated liposarcoma:&lt;br /&gt;
:*Adipocytic or lipoma-like&lt;br /&gt;
:*Inflammatory&lt;br /&gt;
:*Sclerosing&lt;br /&gt;
*Dedifferentiated liposarcoma&lt;br /&gt;
*Myxoid liposarcoma&lt;br /&gt;
*Round cell liposarcoma&lt;br /&gt;
*Pleiomorphic liposarcoma&lt;br /&gt;
&lt;br /&gt;
==References==&lt;br /&gt;
{{reflist|2}}&lt;br /&gt;
[[Category:Disease]]&lt;br /&gt;
[[Category:Types of cancer]]&lt;br /&gt;
[[Category:Oncology]]&lt;br /&gt;
[[Category:Surgery]]&lt;br /&gt;
&lt;br /&gt;
{{WikiDoc Help Menu}}&lt;br /&gt;
{{WikiDoc Sources}}&lt;/div&gt;</summary>
		<author><name>Joao Silva</name></author>
	</entry>
</feed>