Lymphangiosarcoma: Difference between revisions

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{{CMG}} {{AE}} {{Ammu}}
{{CMG}} {{AE}} {{JSS}}


{{SK}}  Stewart-Treves syndrome
{{SK}}  Stewart-Treves syndrome
==Overview==
==Overview==
Lymphangiosarcoma was first discovered by Lowenstein, in 1906, following severe posttraumatic lymphedema of arm for 5 years. Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary [[lymphedema]]. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities. Lymphangiosarcoma may be caused by classical Halstedian radical mastectomy. Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb such as acquired angioedema due to C1 inhibitor deficiency, angioendotheliomatosis, angiolymphoid hyperplasia with eosinophilia, cutaneous [[melanoma]], [[hereditary angioedema]], lymphangiectasia, [[lymphangioma]], and lymphocytoma cutis. On gross pathology, pachydermatous skin, edema, and reddish blue macules or nodules are characteristic findings of lymphangiosarcoma. On microscopic histopathological analysis, proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, hyperchromatism, [[pleomorphism]], mitoses, [[pinocytosis]], intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis are charectoristic findings of lymphangiosarcoma.The prevalence of Stewart-Treves syndrome is approximately 400 worldwide. Common risk factors in the development of lymphangiosarcoma are lymphatic blockage, [[radiotherapy]], mastectomy, cardiovascular diseases, and [[hypertension]].<ref name="pmid19918554">{{cite journal| author=Sepah YJ, Umer M, Qureshi A, Khan S| title=Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report. | journal=Cases J | year= 2009 | volume= 2 | issue=  | pages= 6887 | pmid=19918554 | doi=10.4076/1757-1626-2-6887 | pmc=PMC2769324 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19918554  }} </ref>The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the [[skin]] and subcutaneous tissue. It metastasizes quickly. Findings on [[biopsy]] and ultrastructural histologic studies include proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, hyperchromatism, [[pleomorphism]], [[mitoses]], [[pinocytosis]], intercellular junctions, and cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis. Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.
Lymphangiosarcoma was first discovered by Lowenstein, in 1906. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic [[lymphedema]] of arm. Lymphangiosarcoma is a rare [[malignant]] [[tumor]] which occurs in long-standing cases of primary or secondary [[lymphedema]]. It involves either the upper or lower lymphedemateous extremities but is most common in [[upper extremities]]. Lymphangiosarcoma must be differentiated from other diseases that cause swelling of [[Limb (anatomy)|limb]]. Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported worldwide. Common [[risk factors]] that may lead to the development of lymphangiosarcoma include [[Lymphedema|lymphatic blockage]], [[radiotherapy]], [[mastectomy]], [[cardiovascular diseases]], and [[hypertension]]. The sarcoma first appears as a bruise mark, a purplish discoloration or a [[Tenderness|tender]] [[skin]] [[Nodule (medicine)|nodule]] in the extremity, typically on the [[anterior surface]]. Findings on [[biopsy]] and ultrastructural [[histologic]] studies suggestive of lymphangiosarcoma include proliferating [[vascular]] channels, hyperchromatism, [[pleomorphism]], [[mitoses]], [[pinocytosis]], intercellular junctions, cytoplasmic intermediate filaments, [[Weibel-Palade bodies]], and erythrophagocytosis. [[Amputation]] of the affected limb is the most common approach to the treatment of lymphangiosarcoma.
 
==Historical Perspective==
==Historical Perspective==
*Lymphangiosarcoma was first discovered by Lowenstein, in 1906, following severe posttraumatic lymphedema of arm for 5 years
*Lymphangiosarcoma was first discovered by Lowenstein, in 1906. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic [[lymphedema]] of arm.
*In 1948, postmastectomy lymphedema were first identified in the pathogenesis of lymphangiosarcoma by Fred Stewart and Norman Treves.
*In 1948, Fred Stewart and Norman Treves first identified postmastectomy lymphedema as a precursor condition leading to lymphangiosarcoma.
*In 1960, the first homograft skin transplantation was developed by  1960 to treat diagnose lymphangiosarcoma.
*In 1960, the first [[homograft]] skin [[transplantation]] was developed to treat lymphangiosarcoma.
*In 1979, the concept of local immunodeficiency was first identified in the pathogenesis of lymphangiosarcoma by Schreiber.<ref name="pmid23838488">{{cite journal| author=McKeown DG, Boland PJ| title=Stewart-Treves syndrome: a case report. | journal=Ann R Coll Surg Engl | year= 2013 | volume= 95 | issue= 5 | pages= e80-2 | pmid=23838488 | doi=10.1308/003588413X13629960046110 | pmc=PMC4165172 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23838488  }} </ref>
*In 1979, the concept of local [[immunodeficiency]] was first identified as a possible mechanism leading to the development of lymphangiosarcoma by Schreiber.<ref name="pmid23838488">{{cite journal| author=McKeown DG, Boland PJ| title=Stewart-Treves syndrome: a case report. | journal=Ann R Coll Surg Engl | year= 2013 | volume= 95 | issue= 5 | pages= e80-2 | pmid=23838488 | doi=10.1308/003588413X13629960046110 | pmc=PMC4165172 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23838488  }} </ref>
==Pathophysiology==
==Pathophysiology==
* Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary [[lymphedema]]. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.
* Lymphangiosarcoma is a rare [[Malignant tumors|malignant tumor]] which occurs in cases of chronic [[lymphedema]]<ref name="pmid26777415">{{cite journal| author=Sun S, Chen S, Liu F, Wu H, McHugh J, Bergin IL et al.| title=Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling. | journal=Cancer Cell | year= 2015 | volume= 28 | issue= 6 | pages= 758-772 | pmid=26777415 | doi=10.1016/j.ccell.2015.10.004 | pmc=4828306 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26777415  }} </ref>.
*  When it occurs following mastectomy it is known as Stewart-Treves Syndrome.
* Lymphedema is an abnormal collection of protein-rich fluid in the [[interstitium]] resulting from obstruction of [[Lymphatic system|lymphatic]] drainage<ref name="pmid5094684">{{cite journal| author=Mackenzie DH| title=Lymphangiosarcoma arising in chronic congenital and idiopathic lymphoedema. | journal=J Clin Pathol | year= 1971 | volume= 24 | issue= 6 | pages= 524-9 | pmid=5094684 | doi= | pmc=477086 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5094684  }} </ref>.
* Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs.<ref>Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.</ref>
* Lymphatic obstruction causes an increase in the [[protein]] content of the extravascular [[Tissue (biology)|tissue]], with subsequent retention of water and swelling of the [[soft tissue]].
* Schreiber ''et al.'' proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.<ref>Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310–313</ref><ref>Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves Syndrome: a lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.</ref>
* The increase in the extravascular [[protein]] stimulates proliferation of [[Fibroblast|fibroblasts]], organization of the fluid, and the development of non-pitting [[edema]] of the affected extremity.
*On gross pathology, pachydermatous skin, [[edema]], and reddish blue macules or nodules are characteristic findings of lymphangiosarcoma
* Lymphangiosarcoma arises from the [[endothelial cells]] of [[Lymphatic system|lymphatic vessels]] or [[blood vessels]]<ref name="pmid23372218">{{cite journal| author=Acharya AS, Sulhyan K, Ramteke R, Kunghadkar V| title=Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin. | journal=Indian J Dermatol | year= 2013 | volume= 58 | issue= 1 | pages= 68-70 | pmid=23372218 | doi=10.4103/0019-5154.105314 | pmc=3555379 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23372218  }} </ref>.
*On microscopic histopathological analysis, proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, [[hyperchromatism]], pleomorphism, [[mitoses]], pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis are charectoristic findings of lymphangiosarcoma.
*
*  When it occurs following [[mastectomy]] it is known as Stewart-Treves Syndrome.<ref>Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.</ref>
* The idea of "an immunologically privileged site" was proposed by Schreiber. He explained the concept of chronic [[lymphedema]] leading to local [[immunodeficiency]] which in turn leads to development of [[sarcoma]].  
*On [[gross pathology]], pachydermatous skin, [[edema]], and reddish blue [[Macule|macules]] or [[Nodule (medicine)|nodules]] are characteristic findings of lymphangiosarcoma<ref name="pmid5770234">{{cite journal| author=Barnett WO, Hardy JD, Hendrix JH| title=Lymphangiosarcoma following post-mastectomy lymphedema. | journal=Ann Surg | year= 1969 | volume= 169 | issue= 6 | pages= 960-8 | pmid=5770234 | doi= | pmc=1387587 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5770234  }} </ref>.
*The development of lymphangiosarcoma gradually progresses through 3 stages:<ref name="pmid13413767">{{cite journal| author=LISZAUER S, ROSS RC| title=Lymphangiosarcoma in lymphoedema. | journal=Can Med Assoc J | year= 1957 | volume= 76 | issue= 6 | pages= 475-7 | pmid=13413767 | doi= | pmc=1823629 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13413767  }} </ref><ref name="pmid19918554">{{cite journal| author=Sepah YJ, Umer M, Qureshi A, Khan S| title=Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report. | journal=Cases J | year= 2009 | volume= 2 | issue=  | pages= 6887 | pmid=19918554 | doi=10.4076/1757-1626-2-6887 | pmc=PMC2769324 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19918554  }} </ref><ref name="pmid14903872">{{cite journal| author=FROIO GF, KIRKLAND WG| title=Lymphangiosarcoma in post-mastectomy lymphedema. | journal=Ann Surg | year= 1952 | volume= 135 | issue= 3 | pages= 421-5 | pmid=14903872 | doi= | pmc=1802333 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14903872  }} </ref>.
**'''Stage 1 (prolonged [[lymphedema]]):'''
*** This stage is characterized by extensive [[edema]] that causes the degeneration of [[fat]] and [[collagen]] mainly in the deep part of the [[dermis]].
*** Lymphatic blockade causes persistent [[edema]] resulting in [[fibrosis]] in [[dermis]] and subdermis.
** '''Stage 2 (premalignant [[angiomatosis]]):'''
*** This stage involves multiple foci of small, proliferating channels in the [[dermis]] and subdermis.
*** These [[vessels]] are lined by [[Hyperplasia|hyperplastic]] [[Endothelium|endothelial]] [[cells]].
*** Superficial areas can be seen as [[Bruise|bruises]] or [[Vesicle|vesicles]], whereas deeper areas are seen as areas of [[induration]] and [[Bleeding|haemorrhage]].
** '''Stage 3 ([[malignant]] [[angiosarcoma]]):'''
*** These aggressive [[Tumor|tumors]] develop from areas of premalignant [[angiomatosis]].
*** On microscopic histopathological analysis, proliferating vascular channels, [[hyperchromatism]], pleomorphism, [[mitoses]], [[pinocytosis]], intercellular junctions, [[Cytoplasm|cytoplasmic]] intermediate [[Protein filament|filaments]], Weibel-Palade bodies, and erythrophagocytosis may be found.
==Causes==
==Causes==
* Lymphangiosarcoma may be caused by classical Halstedian radical mastectomy
* Lymphangiosarcoma is caused by chronic [[lymphedema]]<ref name="pmid23372219">{{cite journal| author=Agale SV, Khan WA, Chawlani K| title=Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma. | journal=Indian J Dermatol | year= 2013 | volume= 58 | issue= 1 | pages= 71-3 | pmid=23372219 | doi=10.4103/0019-5154.105315 | pmc=3555380 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23372219  }} </ref><ref name="pmid13155501">{{cite journal| author=HALL-SMITH SP, HABER H| title=Lymphangiosarcoma in postmastectomy lymphoedema. | journal=Proc R Soc Med | year= 1954 | volume= 47 | issue= 3 | pages= 174-5 | pmid=13155501 | doi= | pmc=1918587 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13155501  }} </ref><ref name="pmid13269040">{{cite journal| author=MARSHALL JF| title=Lymphangiosarcoma of the arm following radical mastectomy. | journal=Ann Surg | year= 1955 | volume= 142 | issue= 5 | pages= 871-4 | pmid=13269040 | doi= | pmc=1465017 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13269040  }} </ref><ref name="pmid13383227">{{cite journal| author=KETTLE JH| title=Lymphangiosarcoma following post-mastectomy lymphoedema. | journal=Br Med J | year= 1957 | volume= 1 | issue= 5012 | pages= 193-4 | pmid=13383227 | doi= | pmc=1974216 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13383227  }} </ref>.
* Causes of lymphedema include:
** '''Primary lymphedema'''
*** [[Congenital disorder|Congenital]]
*** Precox ([[adolescence]])
*** Tarda (adulthood)
** '''Secondary lymphedema'''
*** [[Cancer|Malignancy]]
*** Recurrent [[cellulitis]]
*** Connective tissue disease
*** [[Infection]] ([[filariasis]])
*** [[Contact dermatitis]]
*** Lymphatic damage (surgery, [[Radiation therapy|radiation]] therapy, [[Burn|burns]], etc)
==Differentiating Lymphangiosarcoma from other Diseases==
==Differentiating Lymphangiosarcoma from other Diseases==
*Lymphangiosarcoma must be differentiated from other diseases that cause swellling of limb such as:
*Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb such as<ref name="pmid26312725">{{cite journal| author=Pereira ES, Moraes ET, Siqueira DM, Santos MA| title=Stewart Treves Syndrome. | journal=An Bras Dermatol | year= 2015 | volume= 90 | issue= 3 Suppl 1 | pages= 229-31 | pmid=26312725 | doi=10.1590/abd1806-4841.20153685 | pmc=4540559 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26312725  }} </ref><ref name="pmid13608293">{{cite journal| author=RYDELL JR, JENNINGS WK, SMITH ET| title=Postmastectomy lymphedema. | journal=Calif Med | year= 1958 | volume= 89 | issue= 6 | pages= 390-3 | pmid=13608293 | doi= | pmc=1512545 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13608293  }} </ref><ref name="pmid13494640">{{cite journal| author=JANSEY F, SZANTO PB, WRIGHT A| title=Postmastectomy lymphangiosarcoma in elephantiasis chirurgica; Stewart and Treves syndrome. | journal=Q Bull Northwest Univ Med Sch | year= 1957 | volume= 31 | issue= 4 | pages= 301-7 | pmid=13494640 | doi= | pmc=3803612 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13494640  }} </ref>:
:*Acquired angioedema due to C1 inhibitor deficiency
:*[[Angioedema]] due to C1 inhibitor deficiency
:*Angioendotheliomatosis
:*Angioendotheliomatosis
:*Angiolymphoid hyperplasia with eosinophilia
:*[[Angiolymphoid hyperplasia with eosinophilia]]
:*Cutaneous [[melanoma]]
:*Cutaneous [[melanoma]]
:*[[Hereditary angioedema]]
:*[[Hereditary angioedema]]
:*Lymphangiectasia
:*[[Lymphangiectasia]]
:*Lymphangioma
:*[[Lymphangioma]]
:*Lymphocytoma cutis
:*Lymphocytoma cutis
:*[[Metastasis|Metastatic]] [[breast cancer]]
:*[[Melanoma|Malignant melanoma]]
:*[[Kaposi's sarcoma|Kaposi sarcoma]]
:*[[Angiosarcoma]]
The following table differentiates various conditions that may lead to [[Limb (anatomy)|limb]] [[swelling]]:
{|
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Congenital
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Acquired
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! colspan="7" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestations
! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! colspan="1" rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
! colspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Signs
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |CBC
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |LFT
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |ESR/CRP
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hepatosplenomegaly
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lymphadenopathy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |WBC
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hb
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Bacillary angiomatosis]] <ref name="pmid7553576">{{cite journal |vauthors=Tappero JW, Perkins BA, Wenger JD, Berger TG |title=Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus |journal=Clin. Microbiol. Rev. |volume=8 |issue=3 |pages=440–50 |date=July 1995 |pmid=7553576 |pmc=174635 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Human Immunodeficiency Virus (HIV)|HIV]]
* [[Chronic lymphocytic leukemia]]
* Cytotoxic [[chemotherapy]]
* [[Organ transplant|Organ transplantation]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | Any age, usually between 20 -50 years
| style="background:#F5F5F5;" align="center" | Solitary or multiple red, purple, flesh-colored, or colorless [[Papule|papules]]
| style="background:#F5F5F5;" align="center" | ±
| style="background:#F5F5F5;" align="center" | ±
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="left" |
* [[Anorexia]]
* [[Weight loss]]
* [[Abdominal pain]]
* [[Nausea and vomiting]]
* [[Headache]]
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Lobular vascular proliferations of [[Blood vessel|vessels]] lined by plump [[Endothelium|endothelial cells]]
| style="background:#F5F5F5;" align="center" | Clinical manifestation
| style="background:#F5F5F5;" align="left" |
*[[Mental disorder|Psychiatric disorders]]
*[[Personality|Personality changes]]
*[[Seizure]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Arteriovenous malformation]] <ref name="pmid23125071">{{cite journal |vauthors=Whitehead KJ, Smith MC, Li DY |title=Arteriovenous malformations and other vascular malformation syndromes |journal=Cold Spring Harb Perspect Med |volume=3 |issue=2 |pages=a006635 |date=February 2013 |pmid=23125071 |pmc=3552339 |doi=10.1101/cshperspect.a006635 |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Any age
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="left" |
* [[Headache]]
* [[Neurologic diseases|Neurologic deficits]]
* [[Congestive heart failure|Heart failure]]
* [[Macrocephaly]]
| style="background:#F5F5F5;" align="center" |Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" |NA
| style="background:#F5F5F5;" align="center" | [[Imaging]]
| style="background:#F5F5F5;" align="left" |
* [[Hereditary hemorrhagic telangiectasia]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Acroangiodermatitis]]<ref name="pmid17868541">{{cite journal |vauthors=Lugović L, Pusić J, Situm M, Buljan M, Bulat V, Sebetić K, Soldo-Belić A |title=Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports |journal=Acta Dermatovenerol Croat |volume=15 |issue=3 |pages=152–7 |date=2007 |pmid=17868541 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
* Hyperplasia of pre-existing vasculature
* [[Hypertension|HTN]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Any age, more in males
| style="background:#F5F5F5;" align="center" | Purplish-blue to brown [[Papule|papules]] and [[Plaque|plaques]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="left" |
* Paralysed legs
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* [[Hyperkeratosis]]
* Parakeratosis
* [[Acanthosis nigricans|Acanthosis]]
* Mild spongiosis
| style="background:#F5F5F5;" align="center" | Clinical manifesttations
| style="background:#F5F5F5;" align="left" |
* [[Amputation]]
* [[Hemodialysis|Haemodialysis]] patients with [[Arteriovenous fistula|arteriovenous shunts]]
* [[Hepatitis C]]
* [[Venous insufficiency|Chronic venous insufficiency]]
* [[Arteriovenous malformation|AV malformations]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" | [[Angiosarcoma]] <ref name="pmid2734404">{{cite journal |vauthors=Barttelbort SW, Stahl R, Ariyan S |title=Cutaneous angiosarcoma of the face and scalp |journal=Plast. Reconstr. Surg. |volume=84 |issue=1 |pages=55–9 |date=July 1989 |pmid=2734404 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Adults, more in males
| style="background:#F5F5F5;" align="center" |Enlarging [[bruise]], a blue-black [[Nodule (medicine)|nodule]], or an unhealed [[Ulcer|ulceration]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | ↓
| style="background:#F5F5F5;" align="center" | ↓
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Intercellular and intracellular lumina with or without [[Red blood cell|red cells]]
* Intermediate filaments and pinocytotic vesicles in [[cytoplasm]]
* [[Weibel-Palade body|Weibel-Palade bodies]]
| style="background:#F5F5F5;" align="center" | [[Biopsy]]
| style="background:#F5F5F5;" align="center" |NA
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Congenital
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Acquired
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hepatosplenomegaly
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lymphadenopathy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |WBC
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hb
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |LFT
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |ESR/CRP
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Masson's [[hemangioma]] <ref name="pmid22993679">{{cite journal |vauthors=Park KK, Won YS, Yang JY, Choi CS, Han KY |title=Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review |journal=J Korean Neurosurg Soc |volume=52 |issue=1 |pages=52–4 |date=July 2012 |pmid=22993679 |pmc=3440504 |doi=10.3340/jkns.2012.52.1.52 |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" |Rare
| style="background:#F5F5F5;" align="left" |
* Normal
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Papillary fronds lined by proliferating [[endothelium]]
| style="background:#F5F5F5;" align="center" | [[Biopsy]]
| style="background:#F5F5F5;" align="left" |
* [[Hemangioma]]
* [[Pyogenic granuloma|Pyogenic granulomas]]
* [[Lymphangioma overview|Lymphangiomas]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Seborrheic keratosis]] <ref name="pmid18845088">{{cite journal |vauthors=Noiles K, Vender R |title=Are all seborrheic keratoses benign? Review of the typical lesion and its variants |journal=J Cutan Med Surg |volume=12 |issue=5 |pages=203–10 |date=2008 |pmid=18845088 |doi=10.2310/7750.2008.07096 |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* Clonal expansion of a mutated epidermal [[keratinocyte]]
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Any age
| style="background:#F5F5F5;" align="left" |
* Usually [[asymptomatic]]
* Being stuck on the skin surface
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Papillomatous epithelial proliferation containing horn [[Cyst|cysts]]
| style="background:#F5F5F5;" align="center" | Clinical manifestations
| style="background:#F5F5F5;" align="left" |
* [[Dermatosis papulosa nigra]]
* Stucco keratosis
* Melanoacanthoma
* Polypoid lesions
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Systemic lupus erythematosus]] ([[SLE]]) <ref name="pmid22888407">{{cite journal |vauthors=Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P |title=Cutaneous manifestations of systemic lupus erythematosus |journal=Autoimmune Dis |volume=2012 |issue= |pages=834291 |date=2012 |pmid=22888407 |pmc=3410306 |doi=10.1155/2012/834291 |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | More common in female, typically in the 20 to 30 years
| style="background:#F5F5F5;" align="left" |
* [[Erythema]] on the [[Mouth|nasolabial folds]]
* [[Macule|Macular]] or diffusely erythematous in sun-exposed areas
* Discoid rash
| style="background:#F5F5F5;" align="center" | ±
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | ↑
| style="background:#F5F5F5;" align="center" | ±
| style="background:#F5F5F5;" align="center" | ±
| style="background:#F5F5F5;" align="left" |
* [[Weight loss]]
* [[Headache]]
* [[Arthralgia]]
* [[Myalgia]]
* [[Nausea and vomiting|Nausea]]
* [[Dyspepsia]]
* [[Pleuritic chest pain]]
* [[Dyspnea]]
* [[Hematuria]]
| style="background:#F5F5F5;" align="center" | ↑
| style="background:#F5F5F5;" align="center" | ↓
| style="background:#F5F5F5;" align="center" | ↓
| style="background:#F5F5F5;" align="center" |Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* [[Hyperkeratosis]], epidermal [[atrophy]], vacuolar interface [[dermatitis]]
* Thickening of the [[basement membrane]]
* Superficial, [[Perivascular cell|perivascular]], and perifollicular [[Monocyte|mononuclear cell]] inflammatory infiltrate
| style="background:#F5F5F5;" align="center" | Clinical manifestations
| style="background:#F5F5F5;" align="left" |
* [[Raynaud's phenomenon|Raynaud phenomenon]]
* [[Neuropsychiatry|Neuropsychiatric]] symptoms
* [[Pleural effusion]]
* [[Peptic ulcer|Peptic ulcer disease]]
* [[Pericarditis]]
* [[Myocarditis]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Pyogenic]] granuloma <ref name="pmid22434943">{{cite journal |vauthors=Kamal R, Dahiya P, Puri A |title=Oral pyogenic granuloma: Various concepts of etiopathogenesis |journal=J Oral Maxillofac Pathol |volume=16 |issue=1 |pages=79–82 |date=January 2012 |pmid=22434943 |pmc=3303528 |doi=10.4103/0973-029X.92978 |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Physical trauma|Trauma]]
* Hormonal influences
* [[Virus|Viruses]]
* Cytogenetic clonal deletion abnormalities
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | Any age, usually in 20-30 years
| style="background:#F5F5F5;" align="left" |
* Painless red lesion
* Lobular [[capillary hemangioma]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Neutrophilic infiltration
* [[Bleeding|Hemorrhage]]
* [[Necrosis]] of the overlying [[Epidermis (skin)|epidermis]]
| style="background:#F5F5F5;" align="center" | Clinical manifestation
| style="background:#F5F5F5;" align="center" |NA
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Benign lymphangioendothelioma <ref name="pmid10935645">{{cite journal |vauthors=Guillou L, Fletcher CD |title=Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series |journal=Am. J. Surg. Pathol. |volume=24 |issue=8 |pages=1047–57 |date=August 2000 |pmid=10935645 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | +
| style="background:#F5F5F5;" align="center" | Any ages, median age is 50 years
| style="background:#F5F5F5;" align="left" |
* single, slowly expanding patch, [[plaque]], or [[Nodule (medicine)|nodule]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Thin-walled endothelial-lined spaces that are interspersed between strands of [[collagen]]
| style="background:#F5F5F5;" align="center" |[[Biopsy]]
| style="background:#F5F5F5;" align="center" |NA
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Cavernous [[hemangioma]] <ref name="pmid229814">{{cite journal |vauthors=Goldberg RE, Pheasant TR, Shields JA |title=Cavernous hemangioma of the retina. A four-generation pedigree with neurocutaneous manifestations and an example of bilateral retinal involvement |journal=Arch. Ophthalmol. |volume=97 |issue=12 |pages=2321–4 |date=December 1979 |pmid=229814 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="left" |
* [[Idiopathic]]
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Usually in third to fifth decades of life.
| style="background:#F5F5F5;" align="left" |
* Painless, slowly progressive protrusion or bulging of their globe
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | –
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="center" | Nl
| style="background:#F5F5F5;" align="left" |
* Engorged vascular channels, which are tightly knit and separated by [[Fiber|fibrous]] septae
| style="background:#F5F5F5;" align="center" | Clinical manidestation
| style="background:#F5F5F5;" align="left" |
* [[Diplopia]]
* Decreased [[color vision]]
* [[Visual field loss|Visual field deficits]]
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Congenital
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Acquired
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hepatosplenomegaly
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lymphadenopathy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |WBC
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hb
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |LFT
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |ESR/CRP
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|}
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of Stewart-Treves syndrome is approximately 400 worldwide.
 
* In 1962, the incidence of Stewart-Treves syndrome was estimated to be 0.45% in patients who survive at least 5 years after radical mastectomy and 0.03% of patients surviving 10 or more years after radical mastectomy.
=== Prevalence ===
* Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported worldwide.<ref name="pmid199185542">{{cite journal |vauthors=Sepah YJ, Umer M, Qureshi A, Khan S |title=Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report |journal=Cases J |volume=2 |issue= |pages=6887 |date=September 2009 |pmid=19918554 |doi=10.4076/1757-1626-2-6887 |url=}}</ref><ref name="pmid16811066">{{cite journal| author=Mackenzie DH| title=Lymphangiosarcoma. | journal=J Clin Pathol | year= 1972 | volume= 25 | issue= 3 | pages= 273 | pmid=16811066 | doi= | pmc=477281 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16811066  }} </ref>
 
=== Incidence ===
* [[Incidence]] of lymphangiosarcoma in white American women is estimated to be 1.6 per 100,000.<ref name="pmid8000998">{{cite journal |vauthors=Mack TM |title=Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen |journal=Cancer |volume=75 |issue=1 Suppl |pages=211–44 |date=January 1995 |pmid=8000998 |doi= |url=}}</ref>
 
===Age===
===Age===
*Lymphangiosarcoma is more commonly observed among middle-aged or elderly.
*Lymphangiosarcoma is more commonly observed among middle-aged or elderly (mostly between sixth and seventh decade of life which correlates with a higher [[incidence]] of [[breast cancer]].<ref name="pmid11119680">{{cite journal |vauthors=Chung KC, Kim HJ, Jeffers LL |title=Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients |journal=J Hand Surg Am |volume=25 |issue=6 |pages=1163–8 |date=November 2000 |pmid=11119680 |doi=10.1053/jhsu.2000.18490 |url=}}</ref>
===Gender===
===Gender===
*Female are more commonly affected with lymphangiosarcoma than male.
*Female are more commonly affected with lymphangiosarcoma compared to males.<ref name="pmid111196802">{{cite journal |vauthors=Chung KC, Kim HJ, Jeffers LL |title=Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients |journal=J Hand Surg Am |volume=25 |issue=6 |pages=1163–8 |date=November 2000 |pmid=11119680 |doi=10.1053/jhsu.2000.18490 |url=}}</ref>
===Race===
===Race===
*There is no racial predilection for lymphangiosarcoma.
*There is no racial predilection for lymphangiosarcoma.
==Risk Factors==
==Risk Factors==
* Common risk factors in the development of lymphangiosarcoma are lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension.<ref name="pmid19918554">{{cite journal| author=Sepah YJ, Umer M, Qureshi A, Khan S| title=Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report. | journal=Cases J | year= 2009 | volume= 2 | issue= | pages= 6887 | pmid=19918554 | doi=10.4076/1757-1626-2-6887 | pmc=PMC2769324 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19918554 }} </ref>
Common [[risk factors]] in the development of lymphangiosarcoma include:<ref name="pmid19918554" /><ref name="pmid10507781">{{cite journal| author=Cozen W, Bernstein L, Wang F, Press MF, Mack TM| title=The risk of angiosarcoma following primary breast cancer. | journal=Br J Cancer | year= 1999 | volume= 81 | issue= 3 | pages= 532-6 | pmid=10507781 | doi=10.1038/sj.bjc.6690726 | pmc=2362921 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10507781  }} </ref><ref name="pmid16811066">{{cite journal| author=Mackenzie DH| title=Lymphangiosarcoma. | journal=J Clin Pathol | year= 1972 | volume= 25 | issue= 3 | pages= 273 | pmid=16811066 | doi= | pmc=477281 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16811066  }} </ref><ref name="pmid6029576">{{cite journal| author=Danese CA, Grishman E, Dreiling DA| title=Malignant vascular tumors of the lymphedematous extremity. | journal=Ann Surg | year= 1967 | volume= 166 | issue= 2 | pages= 245-53 | pmid=6029576 | doi= | pmc=1477364 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6029576 }} </ref><ref name="pmid50946842">{{cite journal |vauthors=Mackenzie DH |title=Lymphangiosarcoma arising in chronic congenital and idiopathic lymphoedema |journal=J. Clin. Pathol. |volume=24 |issue=6 |pages=524–9 |date=September 1971 |pmid=5094684 |doi= |url=}}</ref><ref name="pmid233722182">{{cite journal |vauthors=Acharya AS, Sulhyan K, Ramteke R, Kunghadkar V |title=Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin |journal=Indian J Dermatol |volume=58 |issue=1 |pages=68–70 |date=January 2013 |pmid=23372218 |doi=10.4103/0019-5154.105314 |url=}}</ref><ref name="pmid17312553">{{cite journal |vauthors=Hulme SA, Bialostocki A, Hardy SL, Tills MR |title=Stewart-Treves syndrome in a congenitally lymphedematous upper limb |journal=Plast. Reconstr. Surg. |volume=119 |issue=3 |pages=1140–1 |date=March 2007 |pmid=17312553 |doi=10.1097/01.prs.0000253459.62819.e2 |url=}}</ref>
* [[Lymphatic]] blockage
* [[Radiation therapy|Radiotherapy]]
* [[Mastectomy]]
* [[Cardiovascular disease|Cardiovascular diseases]]
* [[Hypertension]]
* [[Milroy's Disease|Milroy’s disease]]
* [[Filarial elephantiasis|Filarial]] lymphedema
 
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
* The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
 
*Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcomais approximately less than 5%.
=== Natural History ===
* Lymphangiosarcoma usually develops in patients with a history of chronic [[lymphedema]], after [[mastectomy]] for [[breast cancer]]<ref name="pmid28331699">{{cite journal| author=Taşdemir A, Karaman H, Ünal D, Mutlu H| title=Stewart-Treves Syndrome after Bilateral Mastectomy and Radiotherapy for Breast Carcinoma: Case Report. | journal=J Breast Health | year= 2015 | volume= 11 | issue= 2 | pages= 92-94 | pmid=28331699 | doi=10.5152/tjbh.2015.1604 | pmc=5351494 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28331699  }} </ref><ref name="pmid13623542">{{cite journal| author=TAYLOR GW| title=[Not Available]. | journal=Postgrad Med J | year= 1959 | volume= 35 | issue= 399 | pages= 2-7 | pmid=13623542 | doi= | pmc=2501941 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13623542  }} </ref>.
* If left untreated, chronic [[lymphedema]] leads to severe chronic [[edema]] of the affected extremity.
* The [[skin]] becomes [[Atrophy|atrophic]] and pachydermatous with [[telangiectasias]].
* In the area of [[edema]], a purple patch appears that becomes a [[subcutaneous]] [[Nodule (medicine)|nodule]], sometimes associated with [[bleeding]] and oozing.
* With time multiple bluish-red [[Nodule (medicine)|nodules]] appear with [[ulcer]] formation, surrounded by areas of [[necrosis]].
* [[Metastasis]] appears quickly after [[nodules]] are formed, the most common site for [[metastasis]] being the [[Lung|lungs]].
 
=== Complications ===
* Complications of lymphangiosarcoma include:<ref name="pmid14639083">{{cite journal |vauthors=Armer JM, Radina ME, Porock D, Culbertson SD |title=Predicting breast cancer-related lymphedema using self-reported symptoms |journal=Nurs Res |volume=52 |issue=6 |pages=370–9 |date=2003 |pmid=14639083 |doi= |url=}}</ref><ref name="pmid19289624">{{cite journal |vauthors=Shih YC, Xu Y, Cormier JN, Giordano S, Ridner SH, Buchholz TA, Perkins GH, Elting LS |title=Incidence, treatment costs, and complications of lymphedema after breast cancer among women of working age: a 2-year follow-up study |journal=J. Clin. Oncol. |volume=27 |issue=12 |pages=2007–14 |date=April 2009 |pmid=19289624 |doi=10.1200/JCO.2008.18.3517 |url=}}</ref>
** [[Erysipelas]]
** [[Deep venous thrombosis]] in areas of chronic [[lymphedema]]
** Recurrent [[Infection|infections]]
** [[Bacteremia]]
** [[Malignancy]]
 
=== Prognosis ===
*[[Prognosis]] is generally poor, and the 5 year survival rate of patients with lymphangiosarcoma is less than 5%.<ref name="pmid60295762">{{cite journal |vauthors=Danese CA, Grishman E, Dreiling DA |title=Malignant vascular tumors of the lymphedematous extremity |journal=Ann. Surg. |volume=166 |issue=2 |pages=245–53 |date=August 1967 |pmid=6029576 |pmc=1477364 |doi= |url=}}</ref><ref name="pmid16420866">{{cite journal |vauthors=Echenique-Elizondo M, Tuneu-Valls A, Zubizarreta J, Lobo C |title=[Stewart-Treves syndrome] |language=Spanish; Castilian |journal=Cir Esp |volume=78 |issue=6 |pages=382–4 |date=December 2005 |pmid=16420866 |doi= |url=}}</ref>
== Diagnosis ==
== Diagnosis ==
=== Symptoms ===
=== Symptoms ===
*Symptoms of lymphangiosarcoma may include the following:
[[Symptoms]] of lymphangiosarcoma may include the following:<ref name="pmid27489627">{{cite journal| author=Parthiban R, Kaler AK, Shariff S, Sangeeta M| title=Squamous cell carcinoma arising from congenital lymphedema. | journal=SAGE Open Med Case Rep | year= 2013 | volume= 1 | issue=  | pages= 2050313X13496507 | pmid=27489627 | doi=10.1177/2050313X13496507 | pmc=4857268 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27489627  }} </ref>
:*Bruise mark, a purplish discoloration
*Chronic [[Edema|swelling]] of the affected area
:*Tender skin nodule in the extremity
*Painful [[Nodule (medicine)|nodules]] in the area of swelling
*Non-healing painful [[Ulcer|ulcers]]
*[[Bleeding]] and oozing from the [[ulcers]]
=== Physical Examination ===
=== Physical Examination ===
*Physical examination may be remarkable for:
[[Physical examination]] may be remarkable for:<ref name="pmid20327191">{{cite journal| author=| title=Lymphangiosarcoma in Chronic Lymphedematous Extremities. | journal=Can Med Assoc J | year= 1962 | volume= 87 | issue= 4 | pages= 192 | pmid=20327191 | doi= | pmc=1849463 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20327191  }} </ref><ref name="pmid17859624">{{cite journal| author=McSwain B, Stephenson S| title=Lymphangiosarcoma of the Edematous Extremity. | journal=Ann Surg | year= 1960 | volume= 151 | issue= 5 | pages= 649-56 | pmid=17859624 | doi= | pmc=1613712 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17859624  }} </ref><ref name="pmid26617830">{{cite journal| author=Cui L, Zhang J, Zhang X, Chang H, Qu C, Zhang J et al.| title=Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. | journal=Int J Clin Exp Pathol | year= 2015 | volume= 8 | issue= 9 | pages= 11108-15 | pmid=26617830 | doi= | pmc=4637645 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26617830  }} </ref>
:* Bruise mark
*[[Lymphedema]]: Non-tender and non pitting [[edema]] of the affected area.
:* A purplish discolorization
*Lymphangiosarcoma:
:* Tender skin nodule in the extremity, typically on the anterior surface
**[[Bruise]] mark
:* Ulcer with crusting
**A purplish discolorization
:* Extensive necrosis involving the skin and subcutaneous tissue
**[[Tenderness|Tender]] [[skin]] [[Nodule (medicine)|nodule]] in the extremity, typically on the [[anterior surface]]
**[[Ulcecur|Ulce]]<nowiki/>r with crusting
**Extensive [[necrosis]] involving the [[skin]] and [[subcutaneous tissue]].
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with lymphangiosarcoma.
*Positive staining for [[Laminin, alpha 1|laminin,]] [[CD31]], [[Collagen, type IV, alpha 1|collagen IV,]] and [[vimentin]] are specific for [[angiosarcoma]].<ref name="pmid24604055">{{cite journal| author=Stanczyk M, Gewartowska M| title=Stewart-Treves syndrome. | journal=Indian J Med Res | year= 2014 | volume= 139 | issue= 1 | pages= 179 | pmid=24604055 | doi= | pmc=3994737 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24604055  }} </ref>
===Imaging Findings===
===Imaging Findings===
*[[MRI] is the imaging modality of choice to asses the local extend of lymphangiosarcoma.
*[[MRI]] with intravenous contrast is the imaging modality of choice to asses the local extent of lymphangiosarcoma.
*On [[MRI]], lymphangiosarcoma  is characterized by low signal intensity on T2-weighting.  
*On [[MRI]], lymphangiosarcoma  is characterized by a [[soft tissue]] mass with extension through the sub cutaneous tissue and up to the muscle layer with enhancement.  
*Chest radiography may demonstrate pulmonary metastasis.
*Chest radiography and chest [[CT scan]] may demonstrate [[Lung|pulmonary]] [[metastasis]].
 
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*Lymphangiosarcoma may also be diagnosed by measuring antibodies against factor VIII–related antigen, CD34 antigen, antikeratin antibodies, and positive staining for laminin, CD31, collagen IV, and vimentin.
*Lymphangiosarcoma may also be diagnosed by measuring [[antibodies]] against [[factor VIII]]–related [[antigen]], [[CD34]] [[antigen]], antikeratin [[antibodies]], and positive staining for [[laminin]], [[CD31]], [[collagen]] IV, and [[vimentin]].
*Findings on biopsy and ultrastructural histologic studies include proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, and cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis.
*Findings on [[biopsy]] and ultrastructural [[histologic]] studies include proliferating [[vascular]] channels, hyperchromatism, [[pleomorphism]], [[mitoses]], [[pinocytosis]], intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis.
 
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*The medical therapy of  lymphangiosarcoma is paclitaxel, doxorubicin, ifosfamide, and gemcitabine
*The medical therapy of  lymphangiosarcoma is [[paclitaxel]], [[doxorubicin]], [[ifosfamide]], and [[gemcitabine]].<ref name="pmid24604055">{{cite journal| author=Stanczyk M, Gewartowska M| title=Stewart-Treves syndrome. | journal=Indian J Med Res | year= 2014 | volume= 139 | issue= 1 | pages= 179 | pmid=24604055 | doi= | pmc=3994737 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24604055  }} </ref>
*Treatment of [[lymphedema]] will prevent the development of lymphangiosarcoma.
 
=== Surgery ===
=== Surgery ===
*Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.
*[[Amputation]] of the affected limb is the most common approach to the treatment of lymphangiosarcoma.<ref name="pmid24604055">{{cite journal| author=Stanczyk M, Gewartowska M| title=Stewart-Treves syndrome. | journal=Indian J Med Res | year= 2014 | volume= 139 | issue= 1 | pages= 179 | pmid=24604055 | doi= | pmc=3994737 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24604055  }} </ref>
=== Prevention ===
=== Prevention ===
*Monitoring patients with lymphedema is the primary preventive measure available for lymphangiosarcoma.
*Treatment of lymphedema is the primary preventive measure available for lymphangiosarcoma.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Vascular medicine]]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Synonyms and keywords: Stewart-Treves syndrome

Overview

Lymphangiosarcoma was first discovered by Lowenstein, in 1906. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic lymphedema of arm. Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities. Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb. Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported worldwide. Common risk factors that may lead to the development of lymphangiosarcoma include lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension. The sarcoma first appears as a bruise mark, a purplish discoloration or a tender skin nodule in the extremity, typically on the anterior surface. Findings on biopsy and ultrastructural histologic studies suggestive of lymphangiosarcoma include proliferating vascular channels, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis. Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.

Historical Perspective

  • Lymphangiosarcoma was first discovered by Lowenstein, in 1906. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic lymphedema of arm.
  • In 1948, Fred Stewart and Norman Treves first identified postmastectomy lymphedema as a precursor condition leading to lymphangiosarcoma.
  • In 1960, the first homograft skin transplantation was developed to treat lymphangiosarcoma.
  • In 1979, the concept of local immunodeficiency was first identified as a possible mechanism leading to the development of lymphangiosarcoma by Schreiber.[1]

Pathophysiology

Causes

Differentiating Lymphangiosarcoma from other Diseases

  • Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb such as[14][15][16]:

The following table differentiates various conditions that may lead to limb swelling:

Diseases Etiology Congenital Acquired Demography Clinical manifestations Lab findings Gold standard diagnosis Associated findings
Symptoms Signs CBC LFT ESR/CRP Histopathology
Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt
Bacillary angiomatosis [17] + Any age, usually between 20 -50 years Solitary or multiple red, purple, flesh-colored, or colorless papules ± ± Nl Nl Nl Nl Nl Nl Clinical manifestation
Arteriovenous malformation [18] + Any age Nl + Nl Nl Nl Nl Nl Nl NA Imaging
Acroangiodermatitis[19] Any age, more in males Purplish-blue to brown papules and plaques Nl
  • Paralysed legs
Nl Nl Nl Nl Nl Clinical manifesttations
Angiosarcoma [20] Adults, more in males Enlarging bruise, a blue-black nodule, or an unhealed ulceration Nl Nl Nl Nl Biopsy NA
Diseases Etiology Congenital Acquired Demography Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt LFT ESR/CRP Histopathology Gold standard diagnosis Associated findings
Masson's hemangioma [21] Rare
  • Normal
Nl Nl Nl Nl Nl Nl Biopsy
Seborrheic keratosis [22] + Any age Nl Nl Nl Nl Nl Nl
  • Papillomatous epithelial proliferation containing horn cysts
Clinical manifestations
Systemic lupus erythematosus (SLE) [23] More common in female, typically in the 20 to 30 years ± ± ± Nl Nl Clinical manifestations
Pyogenic granuloma [24]
  • Trauma
  • Hormonal influences
  • Viruses
  • Cytogenetic clonal deletion abnormalities
+ + Any age, usually in 20-30 years + Nl Nl Nl Nl Nl Nl Clinical manifestation NA
Benign lymphangioendothelioma [25] + Any ages, median age is 50 years Nl Nl Nl Nl Nl Nl
  • Thin-walled endothelial-lined spaces that are interspersed between strands of collagen
Biopsy NA
Cavernous hemangioma [26] Usually in third to fifth decades of life.
  • Painless, slowly progressive protrusion or bulging of their globe
Nl Nl Nl Nl Nl Nl
  • Engorged vascular channels, which are tightly knit and separated by fibrous septae
Clinical manidestation
Diseases Etiology Congenital Acquired Demography Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt LFT ESR/CRP Histopathology Gold standard diagnosis Associated findings

Epidemiology and Demographics

Prevalence

  • Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported worldwide.[27][28]

Incidence

  • Incidence of lymphangiosarcoma in white American women is estimated to be 1.6 per 100,000.[29]

Age

  • Lymphangiosarcoma is more commonly observed among middle-aged or elderly (mostly between sixth and seventh decade of life which correlates with a higher incidence of breast cancer.[30]

Gender

  • Female are more commonly affected with lymphangiosarcoma compared to males.[31]

Race

  • There is no racial predilection for lymphangiosarcoma.

Risk Factors

Common risk factors in the development of lymphangiosarcoma include:[8][32][28][33][34][35][36]

Natural History, Complications and Prognosis

Natural History

Complications

Prognosis

  • Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcoma is less than 5%.[41][42]

Diagnosis

Symptoms

Symptoms of lymphangiosarcoma may include the following:[43]

Physical Examination

Physical examination may be remarkable for:[44][45][46]

Laboratory Findings

Imaging Findings

  • MRI with intravenous contrast is the imaging modality of choice to asses the local extent of lymphangiosarcoma.
  • On MRI, lymphangiosarcoma is characterized by a soft tissue mass with extension through the sub cutaneous tissue and up to the muscle layer with enhancement.
  • Chest radiography and chest CT scan may demonstrate pulmonary metastasis.

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

  • Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.[47]

Prevention

  • Treatment of lymphedema is the primary preventive measure available for lymphangiosarcoma.

References

  1. McKeown DG, Boland PJ (2013). "Stewart-Treves syndrome: a case report". Ann R Coll Surg Engl. 95 (5): e80–2. doi:10.1308/003588413X13629960046110. PMC 4165172. PMID 23838488.
  2. Sun S, Chen S, Liu F, Wu H, McHugh J, Bergin IL; et al. (2015). "Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling". Cancer Cell. 28 (6): 758–772. doi:10.1016/j.ccell.2015.10.004. PMC 4828306. PMID 26777415.
  3. Mackenzie DH (1971). "Lymphangiosarcoma arising in chronic congenital and idiopathic lymphoedema". J Clin Pathol. 24 (6): 524–9. PMC 477086. PMID 5094684.
  4. Acharya AS, Sulhyan K, Ramteke R, Kunghadkar V (2013). "Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin". Indian J Dermatol. 58 (1): 68–70. doi:10.4103/0019-5154.105314. PMC 3555379. PMID 23372218.
  5. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
  6. Barnett WO, Hardy JD, Hendrix JH (1969). "Lymphangiosarcoma following post-mastectomy lymphedema". Ann Surg. 169 (6): 960–8. PMC 1387587. PMID 5770234.
  7. LISZAUER S, ROSS RC (1957). "Lymphangiosarcoma in lymphoedema". Can Med Assoc J. 76 (6): 475–7. PMC 1823629. PMID 13413767.
  8. 8.0 8.1 Sepah YJ, Umer M, Qureshi A, Khan S (2009). "Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report". Cases J. 2: 6887. doi:10.4076/1757-1626-2-6887. PMC 2769324. PMID 19918554.
  9. FROIO GF, KIRKLAND WG (1952). "Lymphangiosarcoma in post-mastectomy lymphedema". Ann Surg. 135 (3): 421–5. PMC 1802333. PMID 14903872.
  10. Agale SV, Khan WA, Chawlani K (2013). "Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma". Indian J Dermatol. 58 (1): 71–3. doi:10.4103/0019-5154.105315. PMC 3555380. PMID 23372219.
  11. HALL-SMITH SP, HABER H (1954). "Lymphangiosarcoma in postmastectomy lymphoedema". Proc R Soc Med. 47 (3): 174–5. PMC 1918587. PMID 13155501.
  12. MARSHALL JF (1955). "Lymphangiosarcoma of the arm following radical mastectomy". Ann Surg. 142 (5): 871–4. PMC 1465017. PMID 13269040.
  13. KETTLE JH (1957). "Lymphangiosarcoma following post-mastectomy lymphoedema". Br Med J. 1 (5012): 193–4. PMC 1974216. PMID 13383227.
  14. Pereira ES, Moraes ET, Siqueira DM, Santos MA (2015). "Stewart Treves Syndrome". An Bras Dermatol. 90 (3 Suppl 1): 229–31. doi:10.1590/abd1806-4841.20153685. PMC 4540559. PMID 26312725.
  15. RYDELL JR, JENNINGS WK, SMITH ET (1958). "Postmastectomy lymphedema". Calif Med. 89 (6): 390–3. PMC 1512545. PMID 13608293.
  16. JANSEY F, SZANTO PB, WRIGHT A (1957). "Postmastectomy lymphangiosarcoma in elephantiasis chirurgica; Stewart and Treves syndrome". Q Bull Northwest Univ Med Sch. 31 (4): 301–7. PMC 3803612. PMID 13494640.
  17. Tappero JW, Perkins BA, Wenger JD, Berger TG (July 1995). "Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus". Clin. Microbiol. Rev. 8 (3): 440–50. PMC 174635. PMID 7553576.
  18. Whitehead KJ, Smith MC, Li DY (February 2013). "Arteriovenous malformations and other vascular malformation syndromes". Cold Spring Harb Perspect Med. 3 (2): a006635. doi:10.1101/cshperspect.a006635. PMC 3552339. PMID 23125071.
  19. Lugović L, Pusić J, Situm M, Buljan M, Bulat V, Sebetić K, Soldo-Belić A (2007). "Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports". Acta Dermatovenerol Croat. 15 (3): 152–7. PMID 17868541.
  20. Barttelbort SW, Stahl R, Ariyan S (July 1989). "Cutaneous angiosarcoma of the face and scalp". Plast. Reconstr. Surg. 84 (1): 55–9. PMID 2734404.
  21. Park KK, Won YS, Yang JY, Choi CS, Han KY (July 2012). "Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review". J Korean Neurosurg Soc. 52 (1): 52–4. doi:10.3340/jkns.2012.52.1.52. PMC 3440504. PMID 22993679.
  22. Noiles K, Vender R (2008). "Are all seborrheic keratoses benign? Review of the typical lesion and its variants". J Cutan Med Surg. 12 (5): 203–10. doi:10.2310/7750.2008.07096. PMID 18845088.
  23. Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P (2012). "Cutaneous manifestations of systemic lupus erythematosus". Autoimmune Dis. 2012: 834291. doi:10.1155/2012/834291. PMC 3410306. PMID 22888407.
  24. Kamal R, Dahiya P, Puri A (January 2012). "Oral pyogenic granuloma: Various concepts of etiopathogenesis". J Oral Maxillofac Pathol. 16 (1): 79–82. doi:10.4103/0973-029X.92978. PMC 3303528. PMID 22434943.
  25. Guillou L, Fletcher CD (August 2000). "Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series". Am. J. Surg. Pathol. 24 (8): 1047–57. PMID 10935645.
  26. Goldberg RE, Pheasant TR, Shields JA (December 1979). "Cavernous hemangioma of the retina. A four-generation pedigree with neurocutaneous manifestations and an example of bilateral retinal involvement". Arch. Ophthalmol. 97 (12): 2321–4. PMID 229814.
  27. Sepah YJ, Umer M, Qureshi A, Khan S (September 2009). "Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report". Cases J. 2: 6887. doi:10.4076/1757-1626-2-6887. PMID 19918554.
  28. 28.0 28.1 Mackenzie DH (1972). "Lymphangiosarcoma". J Clin Pathol. 25 (3): 273. PMC 477281. PMID 16811066.
  29. Mack TM (January 1995). "Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen". Cancer. 75 (1 Suppl): 211–44. PMID 8000998.
  30. Chung KC, Kim HJ, Jeffers LL (November 2000). "Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients". J Hand Surg Am. 25 (6): 1163–8. doi:10.1053/jhsu.2000.18490. PMID 11119680.
  31. Chung KC, Kim HJ, Jeffers LL (November 2000). "Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients". J Hand Surg Am. 25 (6): 1163–8. doi:10.1053/jhsu.2000.18490. PMID 11119680.
  32. Cozen W, Bernstein L, Wang F, Press MF, Mack TM (1999). "The risk of angiosarcoma following primary breast cancer". Br J Cancer. 81 (3): 532–6. doi:10.1038/sj.bjc.6690726. PMC 2362921. PMID 10507781.
  33. Danese CA, Grishman E, Dreiling DA (1967). "Malignant vascular tumors of the lymphedematous extremity". Ann Surg. 166 (2): 245–53. PMC 1477364. PMID 6029576.
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