Neurofibroma differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Neurofibroma}}
{{Neurofibroma}}
{{CMG}}; {{AE}}{{S.M.}} {{SC}}
{{CMG}}; {{AE}}{{S.M.}}  
==Overview==
==Overview==
Neurofibroma must be differentiated from [[schwannoma]], [[dermatofibrosarcoma protuberans]] (DFSP), [[ganglioneuroma]], dermal neurotized [[melanocytic nevus]], myxoid [[liposarcoma]], solitary circumscribed neuroma, traumatic [[neuroma]], superficial angiomyxoma, nerve sheath myxoma, malignant peripheral nerve sheath tumor, [[Lipoma|spindle cell lipoma]], [[Leiomyoma]], inflammatory myofibroblastic tumor, and acrochordon.
[[Neurofibroma]] must be [[Differentiate|differentiated]] from [[schwannoma]], [[dermatofibrosarcoma protuberans]] (DFSP), [[ganglioneuroma]], [[dermal]] neurotized [[melanocytic nevus]], myxoid [[liposarcoma]], [[solitary]] circumscribed [[neuroma]], [[Trauma|traumatic]] [[neuroma]], [[Superficial (human anatomy)|superficial]] angiomyxoma, [[nerve sheath]] [[myxoma]], [[malignant peripheral nerve sheath tumor]], [[Lipoma|spindle cell lipoma]], [[leiomyoma]], [[inflammatory]] myofibroblastic [[tumor]], and [[fibroepithelial polyp]].
 
==Differential Diagnosis==
==Differential Diagnosis==
[[Neurofibroma]] must be differentiated from:<ref name="libre">Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2 Accessed on November 17, 2015 </ref><ref>http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/</ref><ref>http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/</ref>
[[Neurofibroma]] must be [[Differentiate|differentiated]] from:
 
* [[Schwannoma]]
* [[Schwannoma]]
* [[Dermatofibrosarcoma protuberans]] (DFSP)
* [[Dermatofibrosarcoma protuberans]] (DFSP)
* [[Ganglioneuroma]]
* [[Ganglioneuroma]]
* Dermal neurotized [[melanocytic nevus]]
* [[Dermal]] neurotized [[melanocytic nevus]]
* Myxoid [[liposarcoma]]
* Myxoid [[liposarcoma]]
* Solitary circumscribed neuroma (palisaded encapsulated neuroma)
* [[Solitary]] circumscribed [[neuroma]]/palisaded [[Encapsulated organisms|encapsulated]] [[neuroma]]
* Traumatic [[neuroma]]
* [[Trauma|Traumatic]] [[neuroma]]
* Superficial angiomyxoma
* [[Superficial]] angiomyxoma
* Nerve sheath myxoma
* [[Nerve sheath]] [[myxoma]]
* Malignant peripheral nerve sheath tumor (MPNST): has marked atypia and increased mitotic activity, may have necrosis
* [[Malignant peripheral nerve sheath tumor|Malignant peripheral nerve sheath tumor (MPNST)]]/[[malignant]] [[schwannoma]]
* [[Lipoma|Spindle cell lipoma]]
* [[Lipoma|Spindle cell lipoma]]
* [[Leiomyoma]]: has cigar-shaped nuclei, S100 negative, positive for [[smooth muscle]] actin and [[desmin]]
* [[Leiomyoma]]
* Inflammatory myofibroblastic tumor: reactive spindle cells in inflammatory background
* [[Inflammatory]] myofibroblastic [[tumor]]
* Acrochordon (aka skin tag or soft [[fibroma]])
* [[Fibroepithelial polyp]]/[[acrochordon]] (aka [[Skin tags|skin tag]] or [[Soft tissue|soft]] [[fibroma]])
{| class="wikitable"
{| class="wikitable"
|+Differentiating neurofibroma from other diseases
|+Differentiating neurofibroma from other diseases
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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Other associated features
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Other associated features
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Neurofibroma'''<ref name="RodriguezFolpe2012">{{cite journal|last1=Rodriguez|first1=Fausto J.|last2=Folpe|first2=Andrew L.|last3=Giannini|first3=Caterina|last4=Perry|first4=Arie|title=Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems|journal=Acta Neuropathologica|volume=123|issue=3|year=2012|pages=295–319|issn=0001-6322|doi=10.1007/s00401-012-0954-z}}</ref><ref name="ChoiKomurov2017">{{cite journal|last1=Choi|first1=Kwangmin|last2=Komurov|first2=Kakajan|last3=Fletcher|first3=Jonathan S.|last4=Jousma|first4=Edwin|last5=Cancelas|first5=Jose A.|last6=Wu|first6=Jianqiang|last7=Ratner|first7=Nancy|title=An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system|journal=Scientific Reports|volume=7|issue=1|year=2017|issn=2045-2322|doi=10.1038/srep43315}}</ref><ref name="LiaoBooker2018">{{cite journal|last1=Liao|first1=Chung-Ping|last2=Booker|first2=Reid C.|last3=Brosseau|first3=Jean-Philippe|last4=Chen|first4=Zhiguo|last5=Mo|first5=Juan|last6=Tchegnon|first6=Edem|last7=Wang|first7=Yong|last8=Clapp|first8=D. Wade|last9=Le|first9=Lu Q.|title=Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis|journal=Journal of Clinical Investigation|volume=128|issue=7|year=2018|pages=2848–2861|issn=0021-9738|doi=10.1172/JCI99424}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MuirNeubauer2001">{{cite journal|last1=Muir|first1=David|last2=Neubauer|first2=Debbie|last3=Lim|first3=Ingrid T.|last4=Yachnis|first4=Anthony T.|last5=Wallace|first5=Margaret R.|title=Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells|journal=The American Journal of Pathology|volume=158|issue=2|year=2001|pages=501–513|issn=00029440|doi=10.1016/S0002-9440(10)63992-2}}</ref><ref name="WilkinsonManson2004">{{cite journal|last1=Wilkinson|first1=Lana M.|last2=Manson|first2=David|last3=Smith|first3=Charles R.|title=Best Cases from the AFIP|journal=RadioGraphics|volume=24|issue=suppl_1|year=2004|pages=S237–S242|issn=0271-5333|doi=10.1148/rg.24si035170}}</ref><ref name="BernthalJones2013">{{cite journal|last1=Bernthal|first1=Nicholas|last2=Jones|first2=Kevin|last3=Monument|first3=Michael|last4=Liu|first4=Ting|last5=Viskochil|first5=David|last6=Randall|first6=R.|title=Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect|journal=Cancers|volume=5|issue=4|year=2013|pages=519–528|issn=2072-6694|doi=10.3390/cancers5020519}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MautnerFriedrich2003">{{cite journal|last1=Mautner|first1=V. F.|last2=Friedrich|first2=R. E.|last3=von Deimling|first3=A.|last4=Hagel|first4=C.|last5=Korf|first5=B.|last6=Knöfel|first6=M. T.|last7=Wenzel|first7=R.|last8=Fünsterer|first8=C.|title=Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma|journal=Neuroradiology|volume=45|issue=9|year=2003|pages=618–625|issn=0028-3940|doi=10.1007/s00234-003-0964-6}}</ref><ref name="ShenHarper1996">{{cite journal|last1=Shen|first1=M H|last2=Harper|first2=P S|last3=Upadhyaya|first3=M|title=Molecular genetics of neurofibromatosis type 1 (NF1).|journal=Journal of Medical Genetics|volume=33|issue=1|year=1996|pages=2–17|issn=1468-6244|doi=10.1136/jmg.33.1.2}}</ref><ref name="RubinGutmann2005">{{cite journal|last1=Rubin|first1=Joshua B.|last2=Gutmann|first2=David H.|title=Neurofibromatosis type 1 — a model for nervous system tumour formation?|journal=Nature Reviews Cancer|volume=5|issue=7|year=2005|pages=557–564|issn=1474-175X|doi=10.1038/nrc1653}}</ref>
| style="background:#DCDCDC;" align="center" + |'''[[Neurofibroma]]'''<ref name="RodriguezFolpe2012">{{cite journal|last1=Rodriguez|first1=Fausto J.|last2=Folpe|first2=Andrew L.|last3=Giannini|first3=Caterina|last4=Perry|first4=Arie|title=Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems|journal=Acta Neuropathologica|volume=123|issue=3|year=2012|pages=295–319|issn=0001-6322|doi=10.1007/s00401-012-0954-z}}</ref><ref name="ChoiKomurov2017">{{cite journal|last1=Choi|first1=Kwangmin|last2=Komurov|first2=Kakajan|last3=Fletcher|first3=Jonathan S.|last4=Jousma|first4=Edwin|last5=Cancelas|first5=Jose A.|last6=Wu|first6=Jianqiang|last7=Ratner|first7=Nancy|title=An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system|journal=Scientific Reports|volume=7|issue=1|year=2017|issn=2045-2322|doi=10.1038/srep43315}}</ref><ref name="LiaoBooker2018">{{cite journal|last1=Liao|first1=Chung-Ping|last2=Booker|first2=Reid C.|last3=Brosseau|first3=Jean-Philippe|last4=Chen|first4=Zhiguo|last5=Mo|first5=Juan|last6=Tchegnon|first6=Edem|last7=Wang|first7=Yong|last8=Clapp|first8=D. Wade|last9=Le|first9=Lu Q.|title=Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis|journal=Journal of Clinical Investigation|volume=128|issue=7|year=2018|pages=2848–2861|issn=0021-9738|doi=10.1172/JCI99424}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MuirNeubauer2001">{{cite journal|last1=Muir|first1=David|last2=Neubauer|first2=Debbie|last3=Lim|first3=Ingrid T.|last4=Yachnis|first4=Anthony T.|last5=Wallace|first5=Margaret R.|title=Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells|journal=The American Journal of Pathology|volume=158|issue=2|year=2001|pages=501–513|issn=00029440|doi=10.1016/S0002-9440(10)63992-2}}</ref><ref name="WilkinsonManson2004">{{cite journal|last1=Wilkinson|first1=Lana M.|last2=Manson|first2=David|last3=Smith|first3=Charles R.|title=Best Cases from the AFIP|journal=RadioGraphics|volume=24|issue=suppl_1|year=2004|pages=S237–S242|issn=0271-5333|doi=10.1148/rg.24si035170}}</ref><ref name="BernthalJones2013">{{cite journal|last1=Bernthal|first1=Nicholas|last2=Jones|first2=Kevin|last3=Monument|first3=Michael|last4=Liu|first4=Ting|last5=Viskochil|first5=David|last6=Randall|first6=R.|title=Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect|journal=Cancers|volume=5|issue=4|year=2013|pages=519–528|issn=2072-6694|doi=10.3390/cancers5020519}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MautnerFriedrich2003">{{cite journal|last1=Mautner|first1=V. F.|last2=Friedrich|first2=R. E.|last3=von Deimling|first3=A.|last4=Hagel|first4=C.|last5=Korf|first5=B.|last6=Knöfel|first6=M. T.|last7=Wenzel|first7=R.|last8=Fünsterer|first8=C.|title=Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma|journal=Neuroradiology|volume=45|issue=9|year=2003|pages=618–625|issn=0028-3940|doi=10.1007/s00234-003-0964-6}}</ref><ref name="ShenHarper1996">{{cite journal|last1=Shen|first1=M H|last2=Harper|first2=P S|last3=Upadhyaya|first3=M|title=Molecular genetics of neurofibromatosis type 1 (NF1).|journal=Journal of Medical Genetics|volume=33|issue=1|year=1996|pages=2–17|issn=1468-6244|doi=10.1136/jmg.33.1.2}}</ref><ref name="RubinGutmann2005">{{cite journal|last1=Rubin|first1=Joshua B.|last2=Gutmann|first2=David H.|title=Neurofibromatosis type 1 — a model for nervous system tumour formation?|journal=Nature Reviews Cancer|volume=5|issue=7|year=2005|pages=557–564|issn=1474-175X|doi=10.1038/nrc1653}}</ref><ref name="Gray1990">{{cite journal|last1=Gray|first1=Mark H.|title=Immunohistochemical Demonstration of Factor XIIIa Expression in Neurofibromas|journal=Archives of Dermatology|volume=126|issue=4|year=1990|pages=472|issn=0003-987X|doi=10.1001/archderm.1990.01670280056009}}</ref>
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Can be sporadic or as a part of [[Neurofibromatosis 1]] and 2
Can be sporadic or as a part of [[Neurofibromatosis 1]] and 2
* ''[[Neurofibromatosis type I|NF1]] [[gene]] located at [[chromosomal]] region [[CCL7|17q11.2]], codes for''[[neurofibromin]]
* ''[[Neurofibromatosis type I|NF1]] [[gene]] located at [[chromosomal]] region [[CCL7|17q11.2]], [[Code|codes]] for''[[neurofibromin]]
* Functional part of [[neurofibromin]] GAP (or [[GTPase-activating proteins|GTPase-activating protein]]) accelerates the [[Conversion (logic)|conversion]] of the active [[GTP-binding protein|GTP]]-bound [[RAS]] to its inactive GDP-[[Bound state|bound]] form
* Functional part of [[neurofibromin]] GAP (or [[GTPase-activating proteins|GTPase-activating protein]]) accelerates the [[Conversion (logic)|conversion]] of the active [[GTP-binding protein|GTP]]-bound [[RAS]] to its inactive GDP-[[Bound state|bound]] form
* Loss of ''[[RAS]]'' [[control]]<nowiki/>leads to increased [[Activity (chemistry)|activity]] of other [[Signaling pathway|signaling pathways]]<nowiki/>including ''[[C-Raf|RAF]]'', ''[[Extracellular signal-regulated kinases|ERK1/2]]'', ''[[Phosphoinositide 3-kinase|PI3K]]'', ''[[PAK1|PAK]], [[MAPK]], [[SCF-complex|SCF]]/[[c-kit]]'' and ''[[Mammalian target of rapamycin|mTOR-S6 kinase]]''
* Loss of ''[[RAS]]'' [[control]]<nowiki/>leads to increased [[Activity (chemistry)|activity]] of other [[Signaling pathway|signaling pathways]]<nowiki/>including ''[[C-Raf|RAF]]'', ''[[Extracellular signal-regulated kinases|ERK1/2]]'', ''[[Phosphoinositide 3-kinase|PI3K]]'', ''[[PAK1|PAK]], [[MAPK]], [[SCF-complex|SCF]]/[[c-kit]]'' and ''[[Mammalian target of rapamycin|mTOR-S6 kinase]]''
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* Uniphasic, low to moderate cellularity
* Uniphasic, low to moderate cellularity
* Non-encapsulated
* No peripheral perineural [[capsule]]
* Random pattern, only rare palisading
* [[Random]] [[pattern]], only [[rare]] palisading
* No well formed verocy bodies
* No well formed verocy bodies
* Cells separated by collagen bundles
* Hypocellular with abundant [[mucinous]]/myxoid [[matrix]] without hypercellular [[Area|areas]]
* Hypocellular with abundant mucinous matrix
* No peripheral perineural capsule
* Frequent [[mast cells]]
* Frequent [[mast cells]]
* Contains neural [[fibroblasts]] and fibrillary collagen
* Contains [[neural]] [[fibroblasts]] and fibrillary or shredded carrot [[collagen]]
* Random proliferation of [[Schwann cells]] and scattered admixed [[axons]]
* [[Random]] [[proliferation]] of [[Schwann cells]] and scattered admixed [[axons]]
* No nevoid cells
* No [[Nevi|nevoid cells]]
* No epithelial component
* No [[epithelial]] component
* Diffuse growth pattern
* [[Diffuse]] [[growth]] [[pattern]]
* Scant cytoplasm
* Scant [[cytoplasm]]
* Wavy cells with buckled nuclei
* Wavy [[spindle cells]] with buckled [[nuclei]]
* Pseudomeissnerian bodies representing specific differentiation may be present
* Pseudomeissnerian [[Body|bodies]] representing specific [[differentiation]] may be present
* Lacks storiform pattern
* Lacks storiform [[pattern]]
Neurofibroma with degenerative atypia ("ancient change") has following microscopic features:
[[Neurofibroma]] with [[degenerative]] [[atypia]] ("ancient [[Change detection|change]]") has following [[microscopic]] [[Features (pattern recognition)|features]]:
* Localized cells with large pleomorphic nuclei, cytoplasmic nuclear inclusions, smudgy chromatin, and inconspicuous nuclei
* [[Localized disease|Localized]] [[Cells (biology)|cells]] with large [[pleomorphic]] [[nuclei]], [[cytoplasmic]] [[nuclear]] [[inclusions]], smudgy [[chromatin]], and inconspicuous [[nuclei]]
* Absent or very low mitotic activity
* Absent or very low [[Mitotic|mitotic activity]]
* Low to moderate cellularity
* Low to moderate cellularity
|Positive for:
|Positive for:
* [[S100A12|S100]]
* [[S100A12|S100]] (weaker)
* Sox10
* [[SOX10]]
* [[Neurofilament]] (and Bielshowsky)
* [[Neurofilament]] (and Bielshowsky)
* [[GFAP]]
* [[GFAP]]
* [[CD34]]
* [[CD34]] (stronger)
* Factor XIIIa
* Factor XIIIa
* [[Calretinin]] (focal)
* [[Calretinin]] (focal)
* MBP ([[myelin]]-[[Basic (chemistry)|basic]] [[protein]])
Negative for:
* EMA (except in plexiform [[Neurofibroma|neurofibromas]])
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* [[Neurofibromatosis type I|Neurofibromatosis 1]]
* [[Neurofibromatosis type I|Neurofibromatosis 1]]
Line 86: Line 90:
* [[Change detection|Changes]] in [[Movement disorder|movement]] ([[clumsiness]] in [[hands]], trouble [[walking]])
* [[Change detection|Changes]] in [[Movement disorder|movement]] ([[clumsiness]] in [[hands]], trouble [[walking]])
* [[Bowel]] [[incontinence]]
* [[Bowel]] [[incontinence]]
* [[Scoliosis]] (an abnormal [[Curvature of spine|curvature of the spine]], if the [[tumor]] creates [[muscular]] [[imbalance]] or erodes [[bones]] of the [[spine]])
* [[Scoliosis]] (an [[abnormal]] [[Curvature of spine|curvature of the spine]], if the [[tumor]] creates [[muscular]] [[imbalance]] or erodes [[bones]] of the [[spine]])
* Following [[symptoms]] may occur with [[genitourinary tract]] involvement (rarely):
* Following [[symptoms]] may occur with [[genitourinary tract]] involvement (rarely):
** [[Urinary tract infection]] (most common clinical manifestation)
** [[Urinary tract infection]] (most common [[clinical]] manifestation)
** [[Urinary retention]]
** [[Urinary retention]]
** [[Urinary frequency]]
** [[Urinary frequency]]
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** Pelvic mass
** Pelvic mass
** [[Hydronephrosis)|Hydronephrosis]]
** [[Hydronephrosis)|Hydronephrosis]]
** [[Urinary incontinence]] (decreased bladder capacity or compliance)
** [[Urinary incontinence]] (decreased [[Urinary bladder|bladder]] capacity or [[compliance]])
** Appears as a focal mass or diffuse bladder wall thickening in case of a plexiform neurofibroma
** [[Appearance|Appears]] as a focal [[mass]] or [[diffuse]] [[Urinary bladder|bladder]] wall thickening in case of a [[plexiform neurofibroma]]
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* Nerve often not identified, incorporates nerve, axons often present in lesion
* [[Nerve]] often not identified, incorporates [[nerve]], [[axons]] often present in [[lesion]]
* Seldom cystic
* Seldom [[cystic]]
* Frequently multiple
* Frequently multiple
* Widespread soft tissue infiltration
* Widespread [[soft tissue]] [[Infiltration (medical)|infiltration]]
* Tends to displace [[Adnexal and skin appendage neoplasms|adnexa]]
* Tends to displace [[Adnexal and skin appendage neoplasms|adnexa]]
* <2cm in diameter
* <2cm in [[diameter]]
* Lacks distinct lobulation
* [[Lack (manque)|Lacks]] [[Distinctive feature|distinct]] [[Lobule|lobulation]]
* Lacks fat
* [[Lack (manque)|Lacks]] [[fat]]
* Affects [[Individual growth|individuals]] between 20-40 [[Year|years]] of [[age]]
* Men and women are [[Equalism|equally]] affected
* [[Plexiform neurofibroma]] are thought to be [[Congenital disorder|congenital]] and occur earlier in [[life]]
|-
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| style="background:#DCDCDC;" align="center" + |'''Schwannoma'''
| style="background:#DCDCDC;" align="center" + |'''[[Schwannoma]]'''<ref>Schwannoma. Dr Tim Luijkx and Dr Sara Wein et al. http://radiopaedia.org/articles/schwannoma</ref><ref name="wiki">Vestibular Schwannoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Vestibular_schwannoma Accessed on October 2 2015</ref><ref name="pmid2612565">{{cite journal |vauthors=Giordano J, Rogers LV |title=Peripherally administered serotonin 5-HT3 receptor antagonists reduce inflammatory pain in rats |journal=[[European Journal of Pharmacology]] |volume=170 |issue=1-2 |pages=83–6 |year=1989 |pmid=2612565 |doi= |url= |issn= |accessdate=2015-11-20}}</ref><ref name="pmid2588243">{{cite journal |vauthors=Kolvenbach H, Lauven PM, Schneider B, Kunath U |title=Repetitive intercostal nerve block via catheter for postoperative pain relief after thoracotomy |journal=[[The Thoracic and Cardiovascular Surgeon]] |volume=37 |issue=5 |pages=273–6 |year=1989 |pmid=2588243 |doi=10.1055/s-2007-1020331 |url=http://www.thieme-connect.com/DOI/DOI?10.1055/s-2007-1020331 |issn= |accessdate=2015-11-20}}</ref><ref name="pmid3735913">{{cite journal |vauthors=Opaleva-Stegantseva VA, Ivanov AG, Gavrilina IA, Khar'kov EI, Ratovskaia VI |title=[Incidence of sudden death cases in acute coronary insufficiency and acute myocardial infarction at the pre-hospital stage in Krasnoyarsk] |language=Russian |journal=[[Kardiologiia]] |volume=26 |issue=5 |pages=23–6 |year=1986 |pmid=3735913 |doi= |url= |issn= |accessdate=2015-11-20}}</ref>
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* Loss of [[Function (biology)|function]] of the [[tumor suppressor gene]] '''[[Merlin (protein)|merlin]]''' (schwannomin)
* Direct [[Genetics (journal)|genetic]] [[Change detection|change]] involving the ''[[NF2 gene|NF2]]'' [[gene]] on [[chromosome 22]]
* Can occur spontaneously
* [[Mutations]] and biallelic inactivation of ''[[SMARCB1]] ([[Spinal cord|spinal]] [[schwannomas]])''
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* Encapsulated
* [[Encapsulated organisms|Encapsulated]]
* Biphasic (majority entirely hypercellular Antoni A & hypocellular Antoni B)
* Aggregates of [[Spindle cells|spindled cells]] with indistinct [[cytoplasm]] and elongated [[nuclei]] with [[Blunt end|blunt]] pointed ends
* Palisading
* Ancient changes may show [[nuclear]] [[pleomorphism]] and occasionally [[nuclear]] [[inclusions]] as well
* Verocay bodies
* Infrequent [[extracellular]] [[collagen]]
* Infrequent extracellular collagen
* [[Biphasic]]: majority entirely, and  compactly hypercellular '''Antoni A''' & myxoid hypocellular '''Antoni B''' [[Area|areas]] (may be absent in small [[tumors]])
* [[Nuclear]] palisading evident around fibrillary [[Process (anatomy)|process]] ('''Verocay bodies''') in [[cellular]] [[Area|areas]]
* Large, irregularly [[Spaced out|spaced]] [[vessels]] prominent in Antoni B [[Area|areas]]
* Narrow, elongated and wavy [[Cells (biology)|cells]] with tapered ends, [[Interspersed repeat|interspersed]] with [[Collagen|collagen fibers]]
* [[Tumor cell|Tumor cells]] with ill defined [[cytoplasm]], [[dense]] [[chromatin]]
* Often displays [[degenerative]] [[nuclear]] [[atypia]] (ancient [[Change detection|change]])
* [[Rare]] [[Mitotic|mitotic figures]]
* [[Blood vessels]] may show gaping [[tortuous]] [[Luminal|lumina]] having thickened hyalinized walls; may have [[thrombi]]
* Dilated [[vessels]] surrounded/invested by [[hemorrhage]]
* Foamy [[macrophages]]
* [[Lymphoid]] aggregates
* Amianthoid [[Fiber|fibers]] or [[collagenous]] spherules: large [[nodular]] [[Mass|masses]] of [[collagen]] with [[Radiating fibers|radiating]] [[Edge detection|edges]]
* No [[axons]] except where [[nerve]] is attached
* [[Malignant]] [[transformation]] may have [[malignant]] [[Epithelioid cell|epithelioid cells]] and rarely shows [[Divergent evolution|divergent]] [[differentiation]] as [[angiosarcoma]]-like [[Area|areas]]
|Positive for:
|Positive for:
* [[S-100]]
* [[S-100]]
* Sox10
* [[SOX10]]
* [[CD34]]
* [[CD56]]
* Podoplanin
* [[CD34]] (weak)
* [[Neurofilament]] (and Bielshowsky)
* [[Neurofilament]] (and Bielshowsky)
* Factor XIIIa (focal)
* Factor XIIIa (focal)
* [[Calretinin]]
* [[Calretinin]]
* [[GFAP]]
* [[GFAP]]
* EMA ([[capsule]]) highlights the perineural [[fibroblasts]]
* [[Laminin]]
* [[Type IV collagen]]
* [[Vimentin]]
* [[CD68]]
Negative for:
* [[Cytokeratin]]
* [[Desmin]]
* [[SMA]]
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* NF-2 associated
* [[Neurofibromatosis type II|NF-2]] associated
* [[Schwannomatosis]]
* [[Carney complex]]
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* [[Upper limbs]]
* [[Head]] and [[neck]] [[area]] ([[oral cavity]], [[Orbit (anatomy)|orbit]] and [[salivary glands]])
* Deeply seated [[tumors]] are mainly in:
** [[Posterior mediastinum]]
** [[Retroperitoneum]]
* [[Posterior]] [[Spine|spinal]] roots
* [[Bone]]
* [[Gastrointestinal tract]]
* [[Pancreas]]
* [[Liver]]
* [[Thyroid]]
* [[Adrenal glands]]
* [[Lymph nodes]]
* [[Penis]] (rarely)
* [[Vulva]] (rarely)
|
|
[[Symptoms]] of [[schwannoma]] depend on the [[Location parameter|location]] of the [[tumor]]:
*Intracranial [[schwannoma]]:
**[[Acoustic neuroma]] (most common):
***[[Sensorineural]] [[hearing]] loss
***[[Vertigo]]
***[[Tinnitus]]
***[[Facial weakness]]
***[[Facial]] [[numbness]] and [[tingling]]
***[[Headaches]]
***[[Dizziness]]
***[[Difficulty swallowing]] and [[hoarseness]]
***[[Taste]] changes
***[[Confusion]]
**[[Trigeminal]] [[schwannoma]]:
***[[Trigeminal nerve]] [[dysfunction]]
**[[Facial nerve]] [[schwannoma]]:
***[[Facial nerve]] [[dysfunction]]
**[[Jugular foramen]] [[schwannoma]]:
***[[Hearing loss]]
***[[Tinnitus]]
***[[Dysphagia]]
***[[Ataxia]]
***[[Hoarseness]]
**[[Hypoglossal nerve|Hypoglossal]] [[schwannomas]]:
***[[Hypoglossal nerve]] [[dysfunction]]
*[[Spine|Spinal]] [[Schwannoma|schwannoma:]]
**[[Back pain]]
**[[Urinary incontinence]]
**[[Urinary retention]]
**[[Clumsiness]]
**[[Weakness]]
**[[Paresthesias]]
*[[Intercostal nerve]] [[schwannoma]]:
**Usually [[asymptomatic]]
*[[Intramuscular]] [[schwannoma]]:
**Painless [[mass]]
|
|
* Nerve often identifiable
* [[Nerve]] often identifiable
* Eccentric to nerve, axons generally absent within lesion
* [[Eccentric Lesion|Eccentric]] to [[nerve]], [[axons]] generally absent within [[lesion]]
* Occasionally cystic
* Occasionally [[cystic]]
* Can [[Causes|cause]] other [[neoplasms]] including:
**[[Meningioma]]
**[[Mesothelioma]]
**[[Glioma|Glioma multiforme]]
**[[Breast Cancer|Breast cancer]]
**[[Colorectal Cancer|Colorectal cancer]]
**[[Renal cell carcinoma|Kidney (clear cell type) carcinoma]]
**[[Hepatocellular carcinoma]]
**[[Prostatic cancer]]
**[[Dermal]] [[cancer]]
 
* [[Affect|Affects]] individuals between 20-50 years of [[age]]
* [[Men]] and women are equally [[Affect|affected]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Palisaded encapsulated neuroma'''
| style="background:#DCDCDC;" align="center" + |'''Palisaded encapsulated [[neuroma]] (PEN) /[[solitary]] circumscribed [[neuroma]]'''<ref name="pmid17414438">{{cite journal| author=Misago N, Inoue T, Narisawa Y| title=Unusual benign myxoid nerve sheath lesion: myxoid palisaded encapsulated neuroma (PEN) or nerve sheath myxoma with PEN/PEN-like features? | journal=Am J Dermatopathol | year= 2007 | volume= 29 | issue= 2 | pages= 160-4 | pmid=17414438 | doi=10.1097/01.dad.0000256688.91974.09 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414438  }} </ref>
|
|
* Spontaneous [[development]]
* [[RET gene|RET]] [[proto-oncogene]] [[genetic mutations]] ([[inherited]] PEN)
|
|
* Solitary
* [[Solitary]] [[dermal]] or [[subcutaneous]] [[tumor]]
* Moderately cellular lesion
* [[Encapsulated organisms|Encapsulated]] by [[perineurium]]
* Club-like [[extension]] in the [[subcutaneous tissue]]
* Moderately [[cellular]] [[lesion]] with [[proliferation]] of [[schwann cells]] and [[axons]]
* [[Nuclear]] palisading may be present
* Rare [[mast cells]]
* Rare [[mast cells]]
* Predominantly composed of [[mast cells]]
* [[Silver staining|'''Silver''' stains]] show the [[axons]] traversing the [[Schwann cells]]
|Positive for:
|Positive for:
* EMA
* EMA
* [[S100A1|S100]] ([[schwann cells]])
* [[Neurofilament]] ([[axons]])
* [[Collagen, type IV, alpha 1|Collagen type IV]]
* EMA ([[perineurium]])
* [[Neuron-Specific Enolase (NSE)|Neuron-specific Enolase]]
* [[CD57]] (Leu-7)
* [[Myelin basic protein|Myelin basic proteins]]
Negative for:
Negative for:
* [[GFAP]]
* [[GFAP]]
|
|
* Positive [[family history]] of [[tumor]] occurrence
* [[Multiple mucosal neuroma syndrome]]
* [[Multiple endocrine neoplasia syndrome]] ([[MEN 2B]])
|90% [[lesions]] affect the [[face]] involving:
* [[Eyelid]]
* [[Nose]]
* [[Oral mucosa]]
Remaining 10% can occur anywhere in [[body]] involving:
* [[Shoulder]]
* [[Arm]]
* [[Hand]]
* [[Foot]]
* [[Glans penis|Glans of penis]]
|
|
* 90% lesions affect the face
* Small, [[solitary]], raised, [[Domes|dome-shaped]], firm, flesh-colored painless [[nodule]] on [[skin]]
* Cosmetic issues due to [[facial]] involvement
* [[Scar]] after [[surgery]]
|
|
|
* [[Benign]] [[tumor]] of the [[nerve fibers]]
* No known familial association
* [[Affect|Affects]] middle [[Age|aged]] people (40-60 years)
* No known [[familial]] [[Association (statistics)|association]]
* [[Affect|Affects]] [[females]] more frequently than [[males]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Traumatic neuroma'''
| style="background:#DCDCDC;" align="center" + |'''[[Traumatic neuroma]]'''<ref name="pmid9745184">{{cite journal| author=Lee EJ, Calcaterra TC, Zuckerbraun L| title=Traumatic neuromas of the head and neck. | journal=Ear Nose Throat J | year= 1998 | volume= 77 | issue= 8 | pages= 670-4, 676 | pmid=9745184 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9745184  }} </ref><ref name="pmid27179535">{{cite journal| author=Hanna SA, Catapano J, Borschel GH| title=Painful pediatric traumatic neuroma: surgical management and clinical outcomes. | journal=Childs Nerv Syst | year= 2016 | volume= 32 | issue= 7 | pages= 1191-4 | pmid=27179535 | doi=10.1007/s00381-016-3109-z | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27179535  }} </ref><ref name="pmid18599222">{{cite journal| author=Foltán R, Klíma K, Spacková J, Sedý J| title=Mechanism of traumatic neuroma development. | journal=Med Hypotheses | year= 2008 | volume= 71 | issue= 4 | pages= 572-6 | pmid=18599222 | doi=10.1016/j.mehy.2008.05.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18599222  }} </ref><ref name="pmid28915703">{{cite journal| author=Yao C, Zhou X, Zhao B, Sun C, Poonit K, Yan H| title=Treatments of traumatic neuropathic pain: a systematic review. | journal=Oncotarget | year= 2017 | volume= 8 | issue= 34 | pages= 57670-57679 | pmid=28915703 | doi=10.18632/oncotarget.16917 | pmc=5593675 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28915703  }} </ref>
|
|
* Tangle of [[neural]] [[Fiber|fibers]] and [[connective tissue]] that [[Development|develops]] following a [[peripheral nerve]] [[injury]]
* Interruption in [[Continuity correction|continuity]] of [[nerve]] [[Causality|causing]] [[wallerian degeneration]] (loss of [[axons]] in [[proximal]] stump and [[retraction]] of [[axons]] in [[distal]] [[Segment (linguistics)|segment]]), followed by exuberant [[regeneration]] of [[nerve]] and [[Formation matrix|formation]] of [[mass]] of [[Schwann cells]], [[axons]] and [[fibrous]] [[Cells (biology)|cells]]
|
|
* Numerous well formed small nerve twigs
* Numerous well formed small [[nerve]] twigs
* Limited soft tissue infiltration
* Limited [[soft tissue]] [[Infiltration (medical)|infiltration]]
* Contains axons in haphazardly arranged nerves
* Contains [[axons]] in haphazardly arranged [[nerves]] within mature [[collagenous]] [[scar]] with entrapped [[smooth muscle]]
|
|Positive for:
|
* [[S100A1|S100]]
* History of trauma or surgery
|
|
|
* History of [[trauma]] to a [[nerve]] (especially during a [[surgery]])
* [[Cone biopsy]] ([[rare]] [[Complications|complication]])
* 55% of [[hysterectomy]] [[patients]] have microneuromas, associated with [[childbirth]]
|Most common [[oral]] [[Location parameter|locations]] are:
* [[Tongue]]
* Near [[mental foramen]] of [[mouth]]
[[Rare|Rarely]] involves:
* [[Head]]
* [[Neck]]
|
|
* Firm, [[oval]], whitish, [[Slow|slowly]] [[Growth|growing]], [[palpable]] [[nodule]] on [[skin]] (no discoloration of [[skin]] on the top of [[nodule]])
* </=2cm in [[Size consistency|size]]
* [[Trauma|Traumatic]] [[neuropathic]] [[pain]] with the presence of a [[Typical set|typical]] [[trigger point]] in the [[area]] of a [[neuroma]]  (especially with the [[pressure]] application) [[Causality|causing]] the [[patient]] to feel burning, stabbing, [[raw]], gnawing or sickening [[sensations]]
* [[Paresthesias|Paresthesia]] over the [[Injured reserve list|injured]] [[area]]
* [[Dysesthesia]] ([[painful]] [[hypersensitivity]] to normal [[light]] [[tactile]] [[Stimulants|stimuli]])
* [[Function (biology)|Functional]] [[impairment]]
* [[Psychological]] [[distress]] (severely decreasing the [[quality of life]])
|Also known as:
* [[Amputation]] [[neuroma]]
* [[Traumatic neuroma|Pseudoneuroma]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Neurotized Melanocytic Nevus'''
| style="background:#DCDCDC;" align="center" + |'''Neurotized [[melanocytic nevus]]'''<ref name="pmid1693815">{{cite journal| author=Gray MH, Smoller BR, McNutt NS, Hsu A| title=Neurofibromas and neurotized melanocytic nevi are immunohistochemically distinct neoplasms. | journal=Am J Dermatopathol | year= 1990 | volume= 12 | issue= 3 | pages= 234-41 | pmid=1693815 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1693815  }} </ref><ref name="pmid22742554">{{cite journal| author=Chen Y, Klonowski PW, Lind AC, Lu D| title=Differentiating neurotized melanocytic nevi from neurofibromas using Melan-A (MART-1) immunohistochemical stain. | journal=Arch Pathol Lab Med | year= 2012 | volume= 136 | issue= 7 | pages= 810-5 | pmid=22742554 | doi=10.5858/arpa.2011-0335-OA | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22742554  }} </ref><ref name="pmid25657396">{{cite journal| author=Singh N, Chandrashekar L, Kar R, Sylvia MT, Thappa DM| title=Neurotized congenital melanocytic nevus resembling a pigmented neurofibroma. | journal=Indian J Dermatol | year= 2015 | volume= 60 | issue= 1 | pages= 46-50 | pmid=25657396 | doi=10.4103/0019-5154.147789 | pmc=4318062 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25657396  }} </ref><ref name="pmid1690969">{{cite journal| author=Gray MH, Smoller BR, McNutt NS, Hsu A| title=Immunohistochemical demonstration of factor XIIIa expression in neurofibromas. A practical means of differentiating these tumors from neurotized melanocytic nevi and schwannomas. | journal=Arch Dermatol | year= 1990 | volume= 126 | issue= 4 | pages= 472-6 | pmid=1690969 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1690969  }} </ref>
|
|
* [[Melanin|Melan]]-A (Mart-1) [[gene]]
* [[Defect]] in [[embryologic]] [[development]] [[Causes|causing]] fast [[proliferation]] [[rate]] of [[melanocytes]] (during first twelve weeks of [[pregnancy]])
|
|
* Superficial classic nevoid [[melanocytes]]
* Neurotized [[Nevus]] is a type of [[mole]] in which  [[melanocytes]] are in the [[dermis]] with accompanying [[fibrosis]]
* Congenital and nested growth patterns
* [[Biphasic]] consisting of [[malignant melanoma]] and mature appearing [[neural]] component
* More abundant cytoplasm
* [[Superficial]] classic [[Nevoid melanoma|nevoid]] [[melanocytes]] (i.e. [[melanocytes]] appear like [[spindle cells]] resembling a [[nerve]]; and hence, called a neurotized [[nevus]])
* [[Congenital]] and nested [[growth]] [[Pattern|patterns]]
* More abundant [[cytoplasm]]
* Tends to surround [[adnexa]]
* Tends to surround [[adnexa]]
* Scattered nests of type A or B [[nevus]] [[Cells (biology)|cells]], surrounded by [[basement membrane]], present in the [[papillary]] [[dermis]] of [[lesions]] (otherwise indistinguishable from [[Neurofibroma|neurofibromas]])
|Positive for:
|Positive for:
* [[S100|S-100]]
* [[S100A1|S-100]]
* MelanA
* MelanA (MART-1)
Negative for:
Negative for:
* Factor XIIIa
* Factor XIIIa
* Leu-7
* [[Glial fibrillary acidic protein|GFAP]]
* [[Myelin basic protein|MBP]]
|
|
* [[Sun exposure]] ([[Ultraviolet light|ultraviolet light)]]
* [[Hormonal]] [[Change detection|changes]] during:
** [[Pregnancy]]
** [[Diabetes]]
* Fair-[[Skin|skinned]] individuals (Caucasians of America and Europe)
* Positive [[family history]] of [[mole]]
|Can occur anywhere in [[body]], mostly involving following [[Area|areas]]:
* [[Head]]
* [[Neck]]
|
|
|
* Slowly growing, [[benign]], [[oval]] or round, well-circumscribed [[macule]], [[papule]] or [[Nodule (medicine)|nodule]]
|
* [[Color]] varies from [[skin]] [[color]] to [[light]] [[brown]] to [[black]]
* Cosmetic concerns
|_
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Cutaneous Myxoma (Superficial angiomyxoma)'''
| style="background:#DCDCDC;" align="center" + |'''[[Cutaneous]] [[myxoma]] ([[Superficial]] angiomyxoma)'''<ref>https://www.sciencedirect.com/topics/medicine-and-dentistry/cutaneous-myxoma</ref><ref name="AlaitiNelson2000">{{cite journal|last1=Alaiti|first1=Samer|last2=Nelson|first2=Fern P.|last3=Ryoo|first3=Jei W.|title=Solitary cutaneous myxoma|journal=Journal of the American Academy of Dermatology|volume=43|issue=2|year=2000|pages=377–379|issn=01909622|doi=10.1067/mjd.2000.101878}}</ref><ref name="Carney1986">{{cite journal|last1=Carney|first1=J. Aidan|title=Cutaneous Myxomas|journal=Archives of Dermatology|volume=122|issue=7|year=1986|pages=790|issn=0003-987X|doi=10.1001/archderm.1986.01660190068018}}</ref><ref name="IidaEgi2019">{{cite journal|last1=Iida|first1=Ken|last2=Egi|first2=Takeshi|last3=Shigi|first3=Masato|last4=Sogabe|first4=Yusuke|last5=Ohashi|first5=Hirotsugu|title=Cutaneous Myxoma of Multiple Lesions|journal=Plastic and Reconstructive Surgery - Global Open|volume=7|issue=2|year=2019|pages=e2040|issn=2169-7574|doi=10.1097/GOX.0000000000002040}}</ref>
|
|
* [[Sporadic Epithelial ovarian tumors|Sporadic]]
* Associated with:
**[[Carney's syndrome]] ([[autosomal dominant]] [[condition]] associated with [[abnormalities]] in [[chromosomes]] 2p and 17q, especially [[mutation]] in the ''[[PRKAR1A|PRKAR1α]]'' [[gene]] on the [[chromosome]] 17q22–q24 [[locus]])
**NAME [[syndrome]]
**LAMB [[syndrome]]
|
|
* [[Spindle]] to stellate cells with oval nuclei
* Predominantly involves [[dermis]] and [[subcutis]]
* 1-5cm in diameter
* Multilobulated, poorly circumscribed
* [[Alcian blue]] positive, and [[hyaluronidase]] sensitive myxoid [[stroma]]/[[acellular]] [[mucin]] pools forming [[cleft]]-like spaces
* Scattered bland [[Stellate cell|stellate]] to [[Spindle cells|spindled cells]] with multiple [[oval]] [[nuclei]]
* [[Rare|Rarely]], [[pleomorphism]], and [[mitotic]] figures seen
* Occasional intranuclear pseudoinclusions
* Many thin-walled small [[blood vessels]]
* Frequent [[neutrophils]]
* Entrapped [[epithelial]] component in 20-30% of cases:
**[[Keratinous]] [[cyst]]
**Thin strands of [[squamous epithelium]]
**Basaloid buds
|Positive for:
|Positive for:
* [[CD34]]
* [[CD34]]
* [[S100|S-100]] (rarely)
* [[Smooth muscle]] [[actin]] (90%)
|
* [[Muscle]] specific [[actin]] (67%)
* Associated with Carney complex
* Factor XIIIa (60%)
* [[Vimentin]]
* [[S100|S-100]] (rarely, 40%)
* Factor VIIIa ([[variable]])
Negative for:
* [[Cytokeratin]]
* [[Desmin]]
* [[Glial fibrillary acidic protein|GFAP]]
* [[ER]]
* [[PR]]
|Associated with '''[[Carney complex|Carney's complex/syndrome]]''' which includes following:
*'''[[Myxomas]]:'''
**[[Cutaneous]]
**[[External ear]]
**[[Heart]]
**[[Breast]] myxoid [[fibroadenoma]]
*'''[[Cutaneous]] [[Melanocytic nevus|melanocytic]] [[lesions]]:'''
**Lentigines
**[[Blue nevus]]
*'''[[Endocrine]] [[hyperplasia]] and [[neoplasia]]''':
**[[Pituitary]]
**[[Thyroid]]
**[[Adrenal cortex]]
**[[Testis]] [[large cell]] calcifying [[Sertoli cell]] [[tumor]]
*'''Psammomatous [[Melanotic cancer|melanotic]] [[schwannoma]]'''
May be associated with '''NAME''' or '''LAMB [[syndrome]]'''
|
|
* [[Trunk]]
* [[Limbs]]
* [[Head]]/[[face]] ([[eyelids]] and [[external ear]] [[Canal (anatomy)|canal]] in [[Carney's syndrome]])
* [[Neck]]
* [[Perineal]]
* [[Nipples]]
* [[Buttocks]]
|
|
* [[Solitary]] or multiple flesh-[[Color|colored]] [[nodules]]
* 1-5cm in [[diameter]]
|
|
* Sometimes, may be the earliest manifestation of [[Carney complex]]
* [[Affect|Affects]] [[men]] more frequently than women
* Involves mostly middle-[[Age|aged]] [[population]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Nerve sheath myxoma'''
| style="background:#DCDCDC;" align="center" + |'''[[Nerve sheath]] [[myxoma]]'''<ref name="pmid16327434">{{cite journal| author=Fetsch JF, Laskin WB, Miettinen M| title=Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 12 | pages= 1615-24 | pmid=16327434 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16327434  }} </ref><ref name="pmid30820132">{{cite journal| author=Yadav SK, Singh S, Sarin N, Naeem R, Pruthi SK| title=Nerve Sheath Myxoma of Scalp: A Rare Site of Presentation. | journal=Int J Trichology | year= 2019 | volume= 11 | issue= 1 | pages= 34-37 | pmid=30820132 | doi=10.4103/ijt.ijt_45_18 | pmc=6385516 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30820132  }} </ref><ref name="pmid26023558">{{cite journal| author=Bhat A, Narasimha A, C V, Vk S| title=Nerve sheath myxoma: report of a rare case. | journal=J Clin Diagn Res | year= 2015 | volume= 9 | issue= 4 | pages= ED07-9 | pmid=26023558 | doi=10.7860/JCDR/2015/10911.5810 | pmc=4437072 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26023558  }} </ref><ref name="pmid17498433">{{cite journal| author=Avninder S, Ramesh V, Vermani S| title=Benign nerve sheath myxoma (myxoid neurothekeoma) in the leg. | journal=Dermatol Online J | year= 2007 | volume= 13 | issue= 2 | pages= 14 | pmid=17498433 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17498433  }} </ref><ref name="pmid24470676">{{cite journal| author=Kim BW, Won CH, Chang SE, Lee MW| title=A case of nerve sheath myxoma on finger. | journal=Indian J Dermatol | year= 2014 | volume= 59 | issue= 1 | pages= 99-101 | pmid=24470676 | doi=10.4103/0019-5154.123526 | pmc=3884944 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24470676  }} </ref><ref name="pmid4091218">{{cite journal| author=Pulitzer DR, Reed RJ| title=Nerve-sheath myxoma (perineurial myxoma). | journal=Am J Dermatopathol | year= 1985 | volume= 7 | issue= 5 | pages= 409-21 | pmid=4091218 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4091218  }} </ref>
|
|
* Unknown [[etiology]]
|
|
* Markedly hypocellular with abundant [[mucopolysaccharides]]
* [[Tumors]] involve the [[dermis]] and/or [[subcutis]]
* Lobulated architecture
* [[Distinctive feature|Distinct]] multinodular/multilobular [[Mass|masses]]
* Markedly hypocellular with abundant myxoid [[stroma]]
* Peripheral [[fibrous]] [[Borderline|border]] made up of [[Collagen, type IV, alpha 1|collagen IV]]
* [[Schwann cells]] may show the following different [[Features (pattern recognition)|features]]:
** [[Cytoplasmic]]-[[nuclear]] [[Invagination|invaginations]]
** Small [[epithelioid]] [[Schwann cells]] in corded, nested, and/or syncytial-like aggregates
** [[Schwann cells]] with a ring-like [[appearance]]
** [[Scattering|Scattered]] [[Spindle cells|spindled]] and [[Stellate cell|stellate]]-shaped [[Schwann cells]]
|Positive for:
|Positive for:
* [[S100|S-100]]
* [[S100A1|S-100]]
|
* [[Glial fibrillary acidic protein]]
|
* [[Neuron-Specific Enolase (NSE)|Neuron specific enolase]]
* [[CD57]]
* [[Epithelial]] [[membrane]] [[antigen]]
* [[CD34]]
|_
|Can occur anywhere in [[body]]:
*Most commonly involves [[extremities]] especially:
**[[Fingers]]
**[[Knees]]
*[[Rare|Rarely]] involves:
**[[Scalp]]/[[head]]
**[[Trunk]]
|
|
* Painless [[skin]] [[mass]] or [[Nodule (medicine)|nodule]]
* Occasionally [[painful]] to [[touch]]
* [[Skin]] over the [[nodule]] is pink, [[Soft tissue|soft]], and usually intact (no [[ulceration]])
* 0.5-2 cm in size
* Cosmetic issue (when present in [[head]] and [[neck]] region)
|
|
* First described by Harkin and Reed in 1969
* Peak [[incidence]] in the fourth decade of [[life]]
* [[Strong]] predilection for the [[extremities]]
* Also known as:
** Classical [[Nerve]] Sheath [[Myxoma]]
** [[Cutaneous]] [[Lobular]] Neuro [[Myxoma]]
** [[Myxomatous]] Perineuroma
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Malignant peripheral nerve sheath tumor'''
| style="background:#DCDCDC;" align="center" + |'''[[Malignant peripheral nerve sheath tumor]] ([[MPNST]])/[[malignant]] [[schwannoma]]'''<ref name="Valeyrie-AllanoreIsmaili2005">{{cite journal|last1=Valeyrie-Allanore|first1=L.|last2=Ismaili|first2=N.|last3=Bastuji-Garin|first3=S.|last4=Zeller|first4=J.|last5=Wechsler|first5=J.|last6=Revuz|first6=J.|last7=Wolkenstein|first7=P.|title=Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1|journal=British Journal of Dermatology|volume=153|issue=1|year=2005|pages=79–82|issn=0007-0963|doi=10.1111/j.1365-2133.2005.06558.x}}</ref><ref name="pmid12011145">{{cite journal| author=Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A| title=Malignant peripheral nerve sheath tumours in neurofibromatosis 1. | journal=J Med Genet | year= 2002 | volume= 39 | issue= 5 | pages= 311-4 | pmid=12011145 | doi= | pmc=1735122 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12011145  }} </ref><ref name="pmid24174807">{{cite journal| author=Panigrahi S, Mishra SS, Das S, Dhir MK| title=Primary malignant peripheral nerve sheath tumor at unusual location. | journal=J Neurosci Rural Pract | year= 2013 | volume= 4 | issue= Suppl 1 | pages= S83-6 | pmid=24174807 | doi=10.4103/0976-3147.116480 | pmc=3808069 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24174807  }} </ref><ref name="pmid17705563">{{cite journal| author=Ferrari A, Bisogno G, Carli M| title=Management of childhood malignant peripheral nerve sheath tumor. | journal=Paediatr Drugs | year= 2007 | volume= 9 | issue= 4 | pages= 239-48 | pmid=17705563 | doi=10.2165/00148581-200709040-00005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17705563  }} </ref><ref name="pmid12632346">{{cite journal| author=Neville H, Corpron C, Blakely ML, Andrassy R| title=Pediatric neurofibrosarcoma. | journal=J Pediatr Surg | year= 2003 | volume= 38 | issue= 3 | pages= 343-6; discussion 343-6 | pmid=12632346 | doi=10.1053/jpsu.2003.50105 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12632346  }} </ref><ref name="ZehouFabre2013">{{cite journal|last1=Zehou|first1=Ouidad|last2=Fabre|first2=Elizabeth|last3=Zelek|first3=Laurent|last4=Sbidian|first4=Emilie|last5=Ortonne|first5=Nicolas|last6=Banu|first6=Eugeniu|last7=Wolkenstein|first7=Pierre|last8=Valeyrie-Allanore|first8=Laurence|title=Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review|journal=Orphanet Journal of Rare Diseases|volume=8|issue=1|year=2013|pages=127|issn=1750-1172|doi=10.1186/1750-1172-8-127}}</ref>
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* 50% arise denovo
* 50% associated with [[neurofibromatosis]] ([[loss of heterozygosity]] of [[p53]] on 17p [[chromosome]])
|
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* Generalized atypia
* Generalized [[atypia]]
* Increased mitotic activity
* Increased [[mitotic]] [[Activity (chemistry)|activity]]
* Diffuse hypercellularity
* [[Diffuse]] hypercellularity
* [[Infiltration (medical)|Infiltrative]] [[growth]]
* [[Pleomorphic]] [[nuclei]]
* [[Area|Areas]] of geographic [[necrosis]] may show [[Divergent synthesis|divergent]] [[differentiation]], with [[tumor]] palisading at edges, resembling [[glioblastoma multiforme]]
* Monomorphic [[Serpentine receptor|serpentine]] [[Cells (biology)|cells]], large [[Gap phenomenon|gaping]] [[vascular]] spaces, [[Perivascular cell|perivascular]] plump [[Tumor cell|tumor cells]]
* May have bizarre [[Cells (biology)|cells]]
* 15% have [[Metaplasticity|metaplastic]] [[cartilage]], [[bone]], and [[muscle]]
* May have [[glandular]] [[differentiation]], if so, presume [[malignant]]
* May have [[melanin]] in [[Tumor cell|tumor cells]], particularly if arise from [[Roots of spinal nerves|spinal nerve roots]] (overlaps with primary [[melanoma]] of [[Nerves|nerves)]]
* Some have no discernable [[Schwann cell|Schwannian]] features at any level
[[Electron microscopy]] shows:
* [[Cell membrane]] infoldings with [[Lamellar bodies|lamellar]] [[Configuration interaction|configuration]], discontinuous [[basal lamina]], conspicuous intercellular junctions, and occasional [[dense]]-[[Core (anatomy)|core]] [[granules]]
 
|Positive for:
|Positive for:
* [[S100|S-100]] (30%)
* [[CD99]]/O13 (86%)
|
* [[S100A1|S-100]] (patchy in 62% cases)
|
* [[CD57]] (55%)
* [[Collagen, type IV, alpha 1|Collagen IV]]
* [[p53]]
* Leu7/[[CD57]] (in [[neurofibroma]]-like [[Area|areas]])
* [[Protein]] [[gene product]] 9.5 (more sensitive than [[S100A1|S100]] but not specific)
In case of [[glandular]] [[differentiation]] ([[malignant]]), positive for:
* [[Keratin]]
* EMA
* [[CEA]]
* [[Chromogranin]]
Negative for:
* [[CD19]]
|Associated with:
* [[NF1]]
May be associated with:
* [[Radiation|Radiations]]
* [[Ganglioneuroma]] ([[Rare|rarely]])
|Bulky deep-seated [[tumor]] usually arising from major [[nerves]] in:
* [[Neck]]
* [[Forearm]]
* [[Lower leg]]
* [[Buttock]]
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* Painless [[swelling]] in [[extremities]] (arms or [[legs]], aka [[peripheral edema]])
* Difficulty [[moving]] the extremity with [[tumor]] ([[Limp|limping]])
* Localized [[Sore|soreness]] in [[tumor]] [[area]]
* [[Neurological]] [[symptoms]]
* [[Pain]] or [[discomfort]]: [[numbness]], burning, or [[Tingling|tingling (pins and needles)]]
* [[Dizziness]]
* [[Loss of balance]]
|
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* Most common frequent [[soft tissue sarcoma]] in the [[pediatrics]] [[population]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Dermatofibrosarcoma protuberans (DFSP)'''
| style="background:#DCDCDC;" align="center" + |'''[[Dermatofibrosarcoma protuberans]] ([[DFSP]])'''
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* t(17,22)(q21;q13) ([[Collagen, type I, alpha 1|collagen type 1 alpha 1(COL1A1)]] [[gene]] and [[Platelet-derived growth factor|platelet derived growth factor  (PDGF)]] [[Beta-1|beta]] [[Chain (sequence)|chain]] [[gene]]), resulting [[fusion protein]] is [[Process (anatomy)|processed]] into mature [[platelet-derived growth factor]] which is a [[Potential|potent]] [[growth factor]]
* Supernumerary ring [[chromosomes]] derived from t(17;22)
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* More cellular
* Usually forms a [[mass]]
* Distinct storiform pattern
* Non circumscribed, highly [[cellular]], tight storiform [[pattern]] ([[Cells (biology)|cells]] [[Radiating fibers|radiating]] in spokes at right angles around a central point that often contains a [[vessel]]) deeply [[Infiltration (medical)|infiltrating]] into [[subcutaneous tissue]] and entraping [[fat cells]] leading to  characteristic honeycomb [[pattern]]
* Usually forms a mass
* [[Area|Areas]] of [[Fascicle|fascicular]] [[growth]] (some [[tumors]])
|Strongly positive for:
* [[Distinctive feature|Distinct]] storiform [[pattern]] may be absent in early [[plaque]] stage
* [[CD34]]
* Monomorphic, thin and [[Spindle cells|spindly cells]] with scant [[eosinophilic]] [[cytoplasm]] and hyperchromatic [[nuclei]] (resembling [[neurofibroma]])
* Numerous [[mitotic]] figures (not atypical ones)
* Non-polarizable and thin [[collagen]]
* Only mild [[pleomorphism]] and focal [[atypia]]
* May coexist with [[giant cell]] fibroblastoma
* Usually no [[Significant figure|significant]] [[pleomorphism]], no / [[rare]] [[histiocytes]], no [[histiocyte]]-like [[Cells (biology)|cells]], no [[foam cells]], no [[giant cells]] or other [[inflammatory cells]]
* '''Variants:''' [[Atrophic]] (depressed [[lesion]]), [[collagenous]] (with [[central]] thick [[collagen]] bundles), [[granular cell]],  myxoid, palisading, [[Pigmented Lesions|pigmented]], and sclerosing
|Positive for:
* [[CD34]] ([[strong]] in 95%)
* [[Vimentin]]
* [[Actin]] (focal)
* ApoD
* [[Bcl-2|Bcl2]]
* NKI-C3
* [[CD99]]
Negative for:
Negative for:
* [[S-100]]
* [[S-100]]
* Factor XIIIa (usually)
* [[Keratin]]
* EMA
* [[S100A1|S100]]
* HMB45
* [[Desmin]]
* [[CD117]]
|_
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* [[Head]]
* Deep [[soft tissue]] of ([[posterior]]) [[neck]]
* [[Trunk]]
* [[Arm|Arms]]
* [[Legs]]
* Doesn't involve [[hands]] and [[feet]]
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* Deep soft tissue of posterior neck
* Begins as a minor firm [[area]] of [[skin]]
|
* 1 to 5 cm in [[diameter]]
* Resembles a [[bruise]], [[birthmark]], or [[pimple]]
* Can become a raised [[Nodule (medicine)|nodule]] after [[growth]]
* May cause [[redness]], open up or [[bleed]]
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* Also called intermediate ([[borderline]]) [[fibrous]] [[histiocytoma]]
* More common in blacks in [[United States|US]]
* Involves [[Adult|adults]] of 20 - 40 years of [[age]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Spindle cell lipoma'''
| style="background:#DCDCDC;" align="center" + |'''[[Spindle cell]] [[lipoma]]'''
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* 16q [[abnormalities]] (usually)
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* Delicate encapsulation
* Delicate [[Encapsulation (pharmacology)|encapsulation]]
* Mostly cases contain fat
* Floret [[cell]] [[Formation matrix|formation]]
* Floret cell formation
* No [[degenerative]] [[atypia]]
* No degenerative atypia
* [[Mixture]] of mature [[adipocytes]] and mildly [[pleomorphic]] bland [[spindle cells]] ([[Paleness|pale]] [[eosinophilic]] [[cytoplasm]] with uniform wavy [[nuclei]] similar to [[neurofibroma]]) in [[mucinous]] / myxoid or [[fibrous]] [[background]] with thick [[collagen]] bundles
* [[Spindle cells]] arranged in short [[fascicles]] with occasional [[nuclear]] palisading
* Hemangiopericytic or angiomatous [[vascular]] [[pattern]] may be seen
* Minimal or no [[fat]]
* Variable [[mast cells]] and [[lymphocytes]]
* No storiform [[pattern]], no lipoblasts, no/[[rare]] [[mitotic]] [[Activity (chemistry)|activity]]
 
|Positive for:
|Positive for:
* CD34 (strongly)
* [[CD34]] ([[Strong|strongly]], [[spindle cells]])
* [[S-100]](stains only fat cells)
* [[Androgen receptors]] in [[men]] and usually women ([[spindle cells]])
|
* [[S-100]]([[Stain|stains]] only [[adipocytes]])
|
[[Spindle cells]] are negative for:
* [[S100A1|S100]]
* [[Desmin]]
|_
|
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* [[Neck]]
* [[Posterior]] upper [[back]]
* [[Shoulder]]
|
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* Multiple well-circumscribed painless [[nodules]] involving several [[body]] parts
|_
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Ganglioneuroma'''<ref name="VasiliadisPapavasiliou2012">{{cite journal|last1=Vasiliadis|first1=K.|last2=Papavasiliou|first2=C.|last3=Fachiridis|first3=D.|last4=Pervana|first4=S.|last5=Michaelides|first5=M.|last6=Kiranou|first6=M.|last7=Makridis|first7=C.|title=Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report|journal=International Journal of Surgery Case Reports|volume=3|issue=11|year=2012|pages=541–543|issn=22102612|doi=10.1016/j.ijscr.2012.07.008}}</ref><ref>https://radiopaedia.org/articles/ganglioneuroma</ref>
| style="background:#DCDCDC;" align="center" + |'''[[Ganglioneuroma]]'''<ref name="VasiliadisPapavasiliou2012">{{cite journal|last1=Vasiliadis|first1=K.|last2=Papavasiliou|first2=C.|last3=Fachiridis|first3=D.|last4=Pervana|first4=S.|last5=Michaelides|first5=M.|last6=Kiranou|first6=M.|last7=Makridis|first7=C.|title=Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report|journal=International Journal of Surgery Case Reports|volume=3|issue=11|year=2012|pages=541–543|issn=22102612|doi=10.1016/j.ijscr.2012.07.008}}</ref><ref>https://radiopaedia.org/articles/ganglioneuroma</ref>
|[[Genes]] involved in the pathogenesis of ganglioneuroma include:
|[[Genes]] involved in the [[pathogenesis]] of [[ganglioneuroma]] include:
* ''MYCN'' [[oncogene]]
* ''MYCN'' [[oncogene]]
* Chromosome 1p36
* [[Chromosome]] 1p36
*Activating RET protooncogene mutation (adrenal ganglioneuromas)  
*[[Activating group|Activating]] [[RET proto-oncogene|RET protooncogene]] [[mutation]] ([[adrenal]] [[Ganglioneuroma|ganglioneuromas]])  
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* Derived from the primordial [[neural crest cells]] (undifferentiated cells of the [[sympathetic nervous system]])
* Derived from the [[Primordial elements|primordial]] [[neural crest cells]] ([[undifferentiated]] [[Cells (biology)|cells]] of the [[sympathetic nervous system]])
* Admixture of [[ganglion cells]], [[schwann cells]], and [[fibrous tissue]]
* Admixture of [[ganglion cells]], [[schwann cells]], and [[fibrous tissue]]
* Doesn't contain [[neuroblasts]], intermediate cells, or [[Mitotic|mitotic figures]]
* Doesn't contain [[neuroblasts]], intermediate [[Cells (biology)|cells]], or [[Mitotic|mitotic figures]]
* Characterized by spindle-shaped cells with cell borders in a fibrillar matrix containing [[ganglion cells]] with large round [[nuclei]], prominent [[nucleoli]], and abundant [[eosinophilic]] cytoplasm
* Characterized by [[spindle]]-shaped [[Cells (biology)|cells]] with [[cell]] borders in a [[Fibrillarin|fibrillar]] [[matrix]] containing [[ganglion cells]] with large round [[nuclei]], prominent [[nucleoli]], and abundant [[eosinophilic]] [[cytoplasm]]
* No significant [[atypia]], necrosis or mitotic activity is present
* No [[Significant figure|significant]] [[atypia]], [[necrosis]] or [[mitotic]] [[Activity (chemistry)|activity]] is present
* Well differentiated neuronal tumors that do not contain immature elements
* Well [[Differentiate|differentiated]] [[neuronal]] [[tumors]] that do not contain immature [[Element|elements]]
*Ganglion cells are mature to mildly dysmorphic:
*[[Ganglion cells]] are mature to mildly [[dysmorphic]]:
**Mature: compact, eosinophilic cytoplasm with distinct cell borders, single eccentric nucleus, prominent nucleolus
**Mature: [[Compact tissue|compact]], [[eosinophilic]] [[cytoplasm]] with [[Distinctive feature|distinct]] [[Cell (biology)|cell]] borders, single [[Eccentricity (mathematics)|eccentric]] [[nucleus]], prominent [[nucleolus]]
**Dysmorphic: single or multiple pyknotic nuclei
**[[Dysmorphic]]: single or multiple [[Pyknosis|pyknotic]] [[nuclei]]
**Vary in distribution and number, may be quite sparse
**Vary in [[Distribution (pharmacology)|distribution]] and [[number]], may be quite [[Sparse coding|sparse]]
**May contain finely granular, gold to brown pigment (lipofuscin or neuromelanin)
**May contain finely [[Granular cell|granular]], [[gold]] to [[brown]] [[pigment]] ([[lipofuscin]] or [[neuromelanin]])
*Schwann cells:
*[[Schwann cells]]:
**Ensheath neuritic processes
**Ensheath neuritic [[Process (anatomy)|processes]]
**Arranged in small intersecting fascicles, separated by loose myxoid stroma
**Arranged in small intersecting [[fascicles]], separated by loose myxoid [[stroma]]
Two histologic subtypes:
Two [[histologic]] subtypes:
*Mature = every ganglion cell is mature
*Mature = every [[ganglion cell]] is mature
*Maturing = minor component of scattered collections of differentiating neuroblasts or maturing ganglion cells (Unlike intermixed subtype of ganglioneuroblastoma, these immature foci do not form distinct microscopic nests)
*Maturing = minor component of scattered collections of [[Differentiating (disease name) from other diseases page|differentiating]] [[neuroblasts]] or maturing [[ganglion cells]] (unlike intermixed subtype of ganglioneuroblastoma, these immature foci do not form [[Distinctive feature|distinct]] [[microscopic]] nests)


*Background may include lobules of mature adipose tissue (especially at periphery of lesion), mast cells, chronic inflammation, dense collagenized stroma
*[[Background]] may include [[lobules]] of mature [[adipose tissue]] (especially at [[periphery]] of [[lesion]]), [[mast cells]], [[Chronic (medicine)|chronic]] [[inflammation]], [[dense]] [[Collagen|collagenized]] [[stroma]]
*Mild variation in cellularity may be present
*Mild variation in [[Cellular|cellularity]] may be present
*Masculinizing ganglioneuroma is an admixture of ganglioneuroma and Leydig cells with crystalloids of Reinke or strands/clusters of cells resembling adrenal cortical cells
*[[Masculinization|Masculinizing]] [[ganglioneuroma]] is an admixture of [[ganglioneuroma]] and [[Leydig cells]] with crystalloids of [[Reinke's edema|Reinke]] or strands/[[Cluster (epidemiology)|clusters]] of [[Cells (biology)|cells]] resembling [[Adrenal cortex|adrenal cortical]] [[Cells (biology)|cells]]
*Electron microscopy:
*[[Electron microscopy]]:
**Mixture of neural bundles and normal appearing ganglion cells with eccentric nuclei and large numbers of cytoplasmic organelles
**[[Mixture]] of [[neural]] bundles and normal [[Appearance|appearing]] [[ganglion cells]] with [[Eccentricity (mathematics)|eccentric]] [[nuclei]] and large [[Number|numbers]] of [[cytoplasmic]] [[organelles]]
|Positive for:
|Positive for:
*Schwann cells/stroma:
*[[Schwann cells]]/[[stroma]]:
**S100
**[[S100A1|S100]]
**Synaptophysin
**[[Synaptophysin]]
**Neurofilament (NF) protein
**[[Neurofilament protein|Neurofilament (NF) protein]]
*Ganglion cells:  
*[[Ganglion cells]]:  
**S100
**[[S100A1|S100]]
**Synaptophysin
**[[Synaptophysin]]
**Chromogranin A
**[[Chromogranin A]]
**NF protein
**[[Neurofilament protein|NF protein]]
**Glial fibrillary acidic protein (GFAP)
**[[Glial fibrillary acidic protein]] ([[GFAP]])
**PGP 9.5
**PGP 9.5
**Type IV collagen
**[[Type IV collagen]]
**Vasoactive intestinal peptide (VIP)
**[[Vasoactive intestinal peptide]] ([[VIP]])
Negative for:
Negative for:
*EMA
*EMA
*Cytokeratin
*[[Cytokeratin]]
*HMB45
*HMB45
*WT1
*[[WT1]]
*CD99
*[[CD99]]
*CD45
*[[CD45]]
*Desmin
*[[Desmin]]
*Myogenic markers (myogenin, MyoD1)
*[[Myogenic]] [[Marker|markers]] ([[myogenin]], MyoD1)
|
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Ganglioneuromas may be associated with:
[[Ganglioneuroma|Ganglioneuromas]] may be associated with:
* [[Multiple endocrine neoplasia]] type IIb ([[mucosal]] ganglioneuromas)
* [[Multiple endocrine neoplasia]] type IIb ([[mucosal]] [[Ganglioneuroma|ganglioneuromas]])
* [[Turner syndrome]]
* [[Turner syndrome]]
* [[Neurofibromatosis type 1]]
* [[Neurofibromatosis type 1]]
|
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Located along distribution of sympathetic nervous system:
Located along [[Distribution (pharmacology)|distribution]] of [[sympathetic nervous system]]:
* Posterior paraspinal [[mediastinum]] (most common)
* [[Posterior]] paraspinal [[mediastinum]] (most common)
* [[Adrenal gland]] (~20-30% of cases)
* [[Adrenal gland]] (~20-30% of cases)
* Paraspinal [[retroperitoneum]] (especially presacral space)
* Paraspinal [[retroperitoneum]] (especially [[presacral space]])
*Cervical and parapharyngeal area in neck
*[[Cervical]] and [[Parapharyngeal space infection|parapharyngeal area]] in [[neck]]
*Urinary bladder
*[[Urinary bladder]]
*Prostate
*[[Prostate]]
*Bone
*[[Bone]]
*Pancreas
*[[Pancreas]]
*Skin
*[[Skin]]
*Orbit
*[[Orbit (anatomy)|Orbit]]
*Paratesticular area
*Paratesticular [[area]]
*Appendix
*[[Appendix]]
*Gastrointestinal tract
*[[Gastrointestinal tract]]
|Symptoms of ganglioneuroma vary depending on the location of tumor, and include the following:
|[[Symptoms]] of [[ganglioneuroma]] vary depending on the [[Location parameter|location]] of [[tumor]], and include the following:
* Mediastinum:
* [[Mediastinum]]:
** Dyspnea  
** [[Dyspnea]]
** Chest pain
** [[Chest pain]]
**Trachea compression
**[[Trachea]] compression
* Retroperitoneum:
* [[Retroperitoneum]]:
** Abdominal pain
** [[Abdominal pain]]
** Bloating
** [[Bloating]]
* Spinal cord:
* [[Spinal cord]]:
** Paresis
** [[Paresis]]
** Pain and numbness/loss of sensation in limbs
** [[Pain]] and [[numbness]]/[[loss of sensation]] in [[limbs]]
Patients with ganglioneuroma may also have [[paraneoplastic syndrome]], which may manifest with:
[[Patients]] with [[ganglioneuroma]] may also have [[paraneoplastic syndrome]], which may manifest with:
* [[Diarrhea]]
* [[Diarrhea]]
* [[Diaphoresis]]
* [[Diaphoresis]]
* [[Hirsuitism|Hirsutism]]
* [[Hirsuitism|Hirsutism]]
*Enlarged clitoris (in females)
*[[Clitoris enlargement|Enlarged clitoris]] (in [[females]])
*High blood pressure
*[[High blood pressure]]
*Sweating
*[[Sweating]]
|
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Ganglioneuromas are included in the ''neuroblastic tumors'' group, which includes:
[[Ganglioneuroma|Ganglioneuromas]] are included in the ''neuroblastic [[tumors]]'' [[Group (sociology)|group]], which includes:
* Ganglioneuroma (benign)
* [[Ganglioneuroma]] ([[benign]])
* Ganglioneuroblastoma (intermediate).
* Ganglioneuroblastoma (intermediate)
* Neuroblastoma (aggressive)
* [[Neuroblastoma]] (aggressive)
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Myxoid liposarcoma'''<ref>{{Cite journal
| style="background:#DCDCDC;" align="center" + |'''Myxoid [[liposarcoma]]'''<ref>{{Cite journal
  | author = [[Khin Thway]], [[Rashpal Flora]], [[Chirag Shah]], [[David Olmos]] & [[Cyril Fisher]]
  | author = [[Khin Thway]], [[Rashpal Flora]], [[Chirag Shah]], [[David Olmos]] & [[Cyril Fisher]]
  | title = Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors
  | title = Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors
Line 428: Line 774:
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'''Well-differentiated liposarcoma''':
'''Well-differentiated liposarcoma''':
* '''Sclerosing [[liposarcoma]]('''distinctive [[stromal]][[Cells (biology)|cells]] distributed across the [[Tissue (biology)|tissue]], associated with lipoblasts filled with multiple [[vacuoles]], and [[collagenous]]<nowiki/>background of fibrillary appearance)
* '''Sclerosing [[liposarcoma]] ('''distinctive [[stromal]] [[Cells (biology)|cells]] distributed across the [[Tissue (biology)|tissue]], associated with lipoblasts filled with multiple [[vacuoles]], and [[collagenous|collageno]]<nowiki/>[[collagenous|us]] background of fibrillary appearance)
* '''[[Adipocyte|Adipocytic]][[liposarcoma]]'''([[adipocytes]] with different [[Cell (biology)|cell]] sizes, hyperchromasia, and [[nuclear]] atypia. [[Fibrous]] [[septa]]<nowiki/>containing hyperchromatic[[stromal cells]]<nowiki/>surrounding [[adipocytes]])
* '''[[Adipocyte|Adipocytic]][[liposarcoma]]''' ([[adipocytes]] with different [[Cell (biology)|cell]] sizes, hyperchromasia, and [[nuclear]] atypia. [[Fibrous]] [[septa|sept]]<nowiki/>[[septa|a]]<nowiki/>containing hyperchromatic [[stromal cells|stromal cel]]<nowiki/>[[stromal cells|ls]] surrounding [[adipocytes]])
* '''[[Inflammatory]][[liposarcoma]]''' (heavy [[Chronic (medical)|chronic]] [[inflammatory]]<nowiki/>infiltrate composed of different lympho-plasmacytic aggregates)
* '''[[Inflammatory]][[liposarcoma]]''' (heavy [[Chronic (medical)|chronic]] [[inflammatory]]<nowiki/>infiltrate composed of different lympho-plasmacytic aggregates)
* '''[[Spindle cells|Spindle cell]][[liposarcoma]]'''([[proliferation]] of [[neural]]-like [[spindle cells]] organized in a [[fibrous]] [[Structure factor|structure]]<nowiki/>containing lipoblasts)
* '''[[Spindle cells|Spindle cell]][[liposarcoma]]'''([[proliferation]] of [[neural]]-like [[spindle cells]] organized in a [[fibrous]] [[Structure factor|structure]]<nowiki/>containing lipoblasts)
'''De-differentiated [[liposarcoma]]''':
'''De-differentiated [[liposarcoma]]''':
* '''Myxoid [[liposarcoma]]'''( non-homogenous [[appearance]] with [[cystic]] and [[solid]]<nowiki/>components)
* '''Myxoid [[liposarcoma]]''' ( non-homogenous [[appearance]] with [[cystic]] and [[solid|soli]]<nowiki/>[[solid|d]] components)
* '''Round [[cell]][[liposarcoma]]''' ( small, round, or [[spindle cells]] with sparse [[Eosinophilia|eosinophilic]] and [[Granular cell|granular]] [[cytoplasm]]<nowiki/>and large [[nuclei]],scattered lipoblasts and [[Area|areas]]<nowiki/>of [[necrosis]])
* '''Round [[cell]][[liposarcoma]]''' (small, round, or [[spindle cells]] with sparse [[Eosinophilia|eosinophilic]] and [[Granular cell|granular]] [[cytoplasm]]<nowiki/>a<nowiki/>nd large [[nuclei]],scattered lipoblasts and [[Area|areas]] <nowiki/>of [[necrosis]])
* '''[[Pleiomorphic]][[liposarcoma]]'''([[pleiomorphic]] [[Cells (biology)|cells]]<nowiki/>with enlarged round to bizarre [[nuclei]])
* '''[[Pleiomorphic|Pleomorphic]][[liposarcoma]]''' ([[pleiomorphic|pleomorphic]] [[Cells (biology)|cells]] <nowiki/>with enlarged round to bizarre [[nuclei]])
|
|
Atypical [[Lipomatous neoplasm|lipomatous tumor]]/well differentiated [[liposarcoma]] is positive for:
Atypical [[Lipomatous neoplasm|lipomatous tumor]]/well differentiated [[liposarcoma]] is positive for:
Line 448: Line 794:
** [[Dioxin]][[Contamination|contaminations]]
** [[Dioxin]][[Contamination|contaminations]]
** [[Arsenic]]
** [[Arsenic]]
** [[Thorium dioxide]]([[Thorotrast]])
** [[Thorium dioxide]] ([[Thorotrast]])
* [[Radiation]] ([[dose]] of 50 GY)
* [[Radiation]] ([[dose]] of 50 GY)
* [[Immunodeficiency]](regional [[acquired]][[immunodeficiency]])
* [[Immunodeficiency]](regional [[acquired]][[immunodeficiency]])
Line 454: Line 800:
*** [[Li-Fraumeni syndrome]]
*** [[Li-Fraumeni syndrome]]
*** [[Neurofibromatosis]]([[NF1]]; [[von Recklinghausen disease]])
*** [[Neurofibromatosis]]([[NF1]]; [[von Recklinghausen disease]])
*** [[Gardner syndrome]]([[Familial adenomatous polyposis]])
*** [[Gardner syndrome]] ([[Familial adenomatous polyposis]])
*** [[Retinoblastoma]]
*** [[Retinoblastoma]]
*** [[Werner syndrome]]
*** [[Werner syndrome]]
Line 473: Line 819:
** [[Abdominal tenderness]]
** [[Abdominal tenderness]]
** [[Abdominal distention]]
** [[Abdominal distention]]
* '''[[Esophageal]] [[liposarcoma]]'''may [[Causes|cause]]:
* '''[[Esophageal]] [[liposarcoma]]''' may [[Causes|cause]]:
** [[Dysphagia]]
** [[Dysphagia]]
** [[Vomiting]]
** [[Vomiting]]
Line 481: Line 827:
* '''[[Bowel]] [[liposarcoma]]''' may cause:
* '''[[Bowel]] [[liposarcoma]]''' may cause:
** [[Gastrointestinal tract|Gastrointestinal]][[bleeding]]
** [[Gastrointestinal tract|Gastrointestinal]][[bleeding]]
* '''[[Mediastinal]] [[liposarcoma]]'''may [[Causes|cause]]:
* '''[[Mediastinal]] [[liposarcoma]]''' may [[Causes|cause]]:
** [[Dyspnea]]
** [[Dyspnea]]
** [[Cough]]
** [[Cough]]
Line 488: Line 834:
|_
|_
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Leiomyoma'''<ref name="pmid7611533">{{cite journal| author=Coffin CM, Watterson J, Priest JR, Dehner LP| title=Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. | journal=Am J Surg Pathol | year= 1995 | volume= 19 | issue= 8 | pages= 859-72 | pmid=7611533 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7611533  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid8847061">{{cite journal| author=Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD| title=Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases. | journal=Histopathology | year= 1995 | volume= 27 | issue= 4 | pages= 313-23 | pmid=8847061 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8847061  }} </ref><ref name="pmid12673560">{{cite journal| author=Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B| title=Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. | journal=Hum Pathol | year= 2003 | volume= 34 | issue= 3 | pages= 253-62 | pmid=12673560 | doi=10.1053/hupa.2003.35 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12673560  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid16967468">{{cite journal| author=Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R et al.| title=Inflammatory myofibroblastic tumors. | journal=J Surg Oncol | year= 2006 | volume= 94 | issue= 5 | pages= 385-91 | pmid=16967468 | doi=10.1002/jso.20516 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16967468  }} </ref><ref name="pmid9606802">{{cite journal| author=Coffin CM, Dehner LP, Meis-Kindblom JM| title=Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations. | journal=Semin Diagn Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 102-10 | pmid=9606802 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9606802  }} </ref><ref name="pmid6336632">{{cite journal| author=Berardi RS, Lee SS, Chen HP, Stines GJ| title=Inflammatory pseudotumors of the lung. | journal=Surg Gynecol Obstet | year= 1983 | volume= 156 | issue= 1 | pages= 89-96 | pmid=6336632 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6336632  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''[[Leiomyoma]]'''<ref name="pmid7611533">{{cite journal| author=Coffin CM, Watterson J, Priest JR, Dehner LP| title=Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. | journal=Am J Surg Pathol | year= 1995 | volume= 19 | issue= 8 | pages= 859-72 | pmid=7611533 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7611533  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid8847061">{{cite journal| author=Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD| title=Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases. | journal=Histopathology | year= 1995 | volume= 27 | issue= 4 | pages= 313-23 | pmid=8847061 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8847061  }} </ref><ref name="pmid12673560">{{cite journal| author=Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B| title=Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. | journal=Hum Pathol | year= 2003 | volume= 34 | issue= 3 | pages= 253-62 | pmid=12673560 | doi=10.1053/hupa.2003.35 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12673560  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid16967468">{{cite journal| author=Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R et al.| title=Inflammatory myofibroblastic tumors. | journal=J Surg Oncol | year= 2006 | volume= 94 | issue= 5 | pages= 385-91 | pmid=16967468 | doi=10.1002/jso.20516 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16967468  }} </ref><ref name="pmid9606802">{{cite journal| author=Coffin CM, Dehner LP, Meis-Kindblom JM| title=Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations. | journal=Semin Diagn Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 102-10 | pmid=9606802 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9606802  }} </ref><ref name="pmid6336632">{{cite journal| author=Berardi RS, Lee SS, Chen HP, Stines GJ| title=Inflammatory pseudotumors of the lung. | journal=Surg Gynecol Obstet | year= 1983 | volume= 156 | issue= 1 | pages= 89-96 | pmid=6336632 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6336632  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
|
|
* Loss of normal [[chromosome]] 1q ([[hereditary]][[leiomyomatosis]])
* Loss of normal [[chromosome]] 1q ([[hereditary]][[leiomyomatosis]])
* [[Renal cell cancer]](HLRCC) [[gene mutation]]
* [[Renal cell cancer]](HLRCC) [[gene mutation]]
|
|
* Prominent [[cellular]][[atypia]]
* Prominent [[cellular]] [[atypia]]
* [[Nuclear]] [[atypia]], including [[nuclear]][[pleomorphism]], hyperchromatism, irregularity in [[nuclear]]<nowiki/>membranes, high [[nuclear]] size, and prominent [[nucleoli]]
* [[Nuclear]] [[atypia]], including [[nuclear]] [[pleomorphism]], hyperchromatism, irregularity in [[nuclear|nucle]]<nowiki/>[[nuclear|ar]]  [[Membrane|membranes]], high [[nuclear]] size, and prominent [[nucleoli]]
* Abundant [[mitoses]], [[mitotic index]] higher than 10 or more per 10 high-power fields
* [[Cigar]]-shaped [[nuclei]]
* [[Area]]<nowiki/>s of [[coagulative necrosis]] ([[tumor cell]][[necrosis]]), always accompanied by [[Cytological|cytologic]] [[atypia]] and brisk [[mitotic]] [[Activity (chemistry)|activity]]
* Abundant [[mitoses]], [[mitotic index]] higher than 10 or more per 10 <nowiki/>high-power fields
* [[Area]]<nowiki/>s of [[coagulative necrosis]] ([[tumor cell]][[necrosis]])
* Pali<nowiki/>sading and extensive [[degenerative]] [[Change|changes]] in the form of hyalinization, [[calcification]], and myxoid changes
* Elon<nowiki/>gated [[Cells (biology)|cells]] with [[eosinophilic]] or occasional [[Fibrillarin|fibrillar]] [[cytoplasm]] with [[Distinctive feature|distinct]] [[cell membranes]]
|
|
Positive for:
Positive for:
Line 532: Line 881:
* [[Retroperitoneum]]
* [[Retroperitoneum]]
* [[Extremities]]
* [[Extremities]]
* Large [[vessels]]([[inferior vena cava]], [[saphenous vein]], [[femoral vein]], [[pulmonary artery]], [[femoral artery]])
* Large [[vessels]] ([[inferior vena cava]], [[saphenous vein]], [[femoral vein]], [[pulmonary artery]], [[femoral artery]])
* [[Superficial]] or deep [[Soft tissue|soft tissues]]
* [[Superficial]] or deep [[Soft tissue|soft tissues]]
* [[Bone]]
* [[Bone]]
Line 568: Line 917:
* [[Spindle cells]]<nowiki/>arranged in short [[fascicles]] with a focal storiform (whorled or cartwheel-like) architecture
* [[Spindle cells]]<nowiki/>arranged in short [[fascicles]] with a focal storiform (whorled or cartwheel-like) architecture
* [[Spindle cells]] show features of [[fibroblasts]]<nowiki/>and [[myofibroblasts]]
* [[Spindle cells]] show features of [[fibroblasts]]<nowiki/>and [[myofibroblasts]]
* Variably dense, chronic, mixed [[polymorphic]] infiltrateof [[Mononuclear cells|mononuclear]][[inflammatory]] ([[plasma cells]] and [[lymphocytes]], [[histiocytes]], [[neutrophils]], and occasional [[eosinophils]])
* Variably dense, chronic, mixed [[polymorphic]] infiltrateof [[Mononuclear cells|mononuclear]][[inflammatory]] [[Cells (biology)|cells]] ([[plasma cells]] and [[lymphocytes]], [[histiocytes]], [[neutrophils]], and occasional [[eosinophils]])
* [[Histiocytes]] have [[multinucleated]] forms with finely vacuolated [[cytoplasmic]] [[lipid]]<nowiki/>droplets
* [[Histiocytes]] have [[multinucleated]] forms with finely vacuolated [[cytoplasmic]] [[lipid]]<nowiki/>droplets
* [[Plasma cells]] with [[cytoplasmic]] [[Russell bodies]] ([[Globular protein|globular]][[cytoplasmic]][[inclusions]] of [[immunoglobulin]]) and [[polyclonal]] pattern of [[light chain]] [[expression]]
* [[Plasma cells]] with [[cytoplasmic]] [[Russell bodies]] ([[Globular protein|globular]][[cytoplasmic]][[inclusions]] of [[immunoglobulin]]) and [[polyclonal]] pattern of [[light chain]] [[expression]]
Line 655: Line 1,004:
* Atypical myfibroblastic [[tumor]]
* Atypical myfibroblastic [[tumor]]
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Acrochordon'''
| style="background:#DCDCDC;" align="center" + |'''[[Fibroepithelial polyp]]/[[Acrochordon]]'''<ref name="pmid30997841">{{cite journal| author=Cukic O, Jovanovic MB| title=Large Fibroepithelial Polyp of the Palatine Tonsil. | journal=Ear Nose Throat J | year= 2019 | volume=  | issue=  | pages= 145561319841203 | pmid=30997841 | doi=10.1177/0145561319841203 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30997841  }} </ref><ref name="pmid30916216">{{cite journal| author=Vatansever M, Dinç E, Dursun Ö, Oktay ÖÖ, Arpaci R| title=Atypical presentation of fibroepithelial polyp: a report of two cases. | journal=Arq Bras Oftalmol | year= 2019 | volume=  | issue=  | pages=  | pmid=30916216 | doi=10.5935/0004-2749.20190050 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30916216  }} </ref><ref name="pmid30864355">{{cite journal| author=Rexhepi M, Trajkovska E, Besimi F, Rufati N| title=Giant Fibroepithelial Polyp of Vulva: A Case Report and Review of Literature. | journal=Pril (Makedon Akad Nauk Umet Odd Med Nauki) | year= 2018 | volume= 39 | issue= 2-3 | pages= 127-130 | pmid=30864355 | doi=10.2478/prilozi-2018-0051 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30864355  }} </ref><ref name="pmid30778021">{{cite journal| author=Jabbour J, Chappell JR, Busby M, McCubbery NW, Brown DF, Park SJK et al.| title=Glottic Obstruction from Fibroepithelial Polyp. | journal=Am J Case Rep | year= 2019 | volume= 20 | issue=  | pages= 219-223 | pmid=30778021 | doi=10.12659/AJCR.914907 | pmc=6388646 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30778021  }} </ref><ref name="pmid30448831">{{cite journal| author=Hong P, Cai Y, Li Z, Fan S, Yang K, Hao H et al.| title=Modified Laparoscopic Partial Ureterectomy for Adult Ureteral Fibroepithelial Polyp: Technique and Initial Experience. | journal=Urol Int | year= 2019 | volume= 102 | issue= 1 | pages= 13-19 | pmid=30448831 | doi=10.1159/000494804 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30448831  }} </ref><ref name="pmid30426076">{{cite journal| author=Uçar M, Baş E, Akkoç A, Topçuoğlu M| title=Fibroepithelial Polyp of the Ureter: A Rare Cause of Hydronephrosis. | journal=J Endourol Case Rep | year= 2018 | volume= 4 | issue= 1 | pages= 166-168 | pmid=30426076 | doi=10.1089/cren.2018.0031 | pmc=6225073 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30426076  }} </ref><ref name="pmid30425926">{{cite journal| author=Chaker K, Rhouma SB, Daly KM, Zehani A, Bibi M, Chehida MAB et al.| title=Benign fibroepithelial polyp of the ureter: A case report. | journal=Urol Case Rep | year= 2019 | volume= 22 | issue=  | pages= 52-53 | pmid=30425926 | doi=10.1016/j.eucr.2018.10.019 | pmc=6226574 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30425926  }} </ref><ref name="pmid30421620">{{cite journal| author=Hajji F, Moufid K, Ghoundale O, Touiti D| title=A rare case of successful endoscopic management of a fibroepithelial polyp with intussusception of the ureter and periodic prolapse into bladder. | journal=Ann R Coll Surg Engl | year= 2019 | volume= 101 | issue= 2 | pages= e66-e70 | pmid=30421620 | doi=10.1308/rcsann.2018.0198 | pmc=6351868 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30421620  }} </ref><ref name="pmid30401014">{{cite journal| author=Lee H, Sade I, Gilani S, Zhong M, Lombardo G| title=A Giant Fibroepithelial Polyp of the Small Bowel Associated with High-Grade Obstruction. | journal=Am Surg | year= 2018 | volume= 84 | issue= 7 | pages= e210-e211 | pmid=30401014 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30401014  }} </ref><ref name="pmid30319938">{{cite journal| author=Chaker K| title=Benign fibroepithelial polyp of the ureter: A case report. | journal=Urol Case Rep | year= 2019 | volume= 22 | issue=  | pages= 15-16 | pmid=30319938 | doi=10.1016/j.eucr.2018.09.021 | pmc=6180234 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30319938  }} </ref><ref name="pmid30009441">{{cite journal| author=Lozano-Peña AK, Lamadrid-Zertuche AC, Ocampo-Candiani J| title=Giant fibroepithelial polyp of the vulva. | journal=Australas J Dermatol | year= 2019 | volume= 60 | issue= 1 | pages= 70-71 | pmid=30009441 | doi=10.1111/ajd.12886 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30009441  }} </ref><ref name="pmid29883781">{{cite journal| author=Eckstein M, Agaimy A, Woenckhaus J, Winter A, Bittmann I, Janzen J et al.| title=DICER1 mutation-positive giant botryoid fibroepithelial polyp of the urinary bladder mimicking embryonal rhabdomyosarcoma. | journal=Hum Pathol | year= 2019 | volume= 84 | issue=  | pages= 1-7 | pmid=29883781 | doi=10.1016/j.humpath.2018.05.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29883781  }} </ref><ref name="pmid29843497">{{cite journal| author=Akdere H, Çevik G| title=Rare Fibroepithelial Polyp Extending Along the Ureter: A Case Report | journal=Balkan Med J | year= 2018 | volume= 35 | issue= 3 | pages= 275-277 | pmid=29843497 | doi=10.4274/balkanmedj.2017.1537 | pmc=5981127 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29843497  }} </ref><ref name="pmid29800931">{{cite journal| author=Ballard DH, Rove KO, Coplen DE, Chen TY, Hulett Bowling RL| title=Fibroepithelial polyp causing urethral obstruction: Diagnosis by cystourethrogram. | journal=Clin Imaging | year= 2018 | volume= 51 | issue=  | pages= 164-167 | pmid=29800931 | doi=10.1016/j.clinimag.2018.05.009 | pmc=6404776 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29800931  }} </ref><ref name="pmid29574427">{{cite journal| author=Amin A, Amin Z, Al Farsi AR| title=Septic presentation of a giant fibroepithelial polyp of the vulva. | journal=BMJ Case Rep | year= 2018 | volume= 2018 | issue=  | pages=  | pmid=29574427 | doi=10.1136/bcr-2017-222789 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29574427  }} </ref><ref name="pmid29487969">{{cite journal| author=Gupta R, Smita S, Sinha R, Sinha N, Sinha L| title=Giant fibroepithelial polyp of the thigh and retroperitoneal fibromatosis in a young woman: a rare case. | journal=Skeletal Radiol | year= 2018 | volume= 47 | issue= 9 | pages= 1299-1304 | pmid=29487969 | doi=10.1007/s00256-018-2904-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29487969  }} </ref><ref name="pmid29200704">{{cite journal| author=Rajeesh Mohammed PK, Choudhury BK, Dalai RP, Rana V| title=Fibroepithelial Polyp with Sebaceous Hyperplasia: A Case Report. | journal=Indian J Med Paediatr Oncol | year= 2017 | volume= 38 | issue= 3 | pages= 404-406 | pmid=29200704 | doi=10.4103/ijmpo.ijmpo_124_17 | pmc=5686997 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29200704  }} </ref><ref name="pmid28791276">{{cite journal| author=Lee MH, Hwang JY, Lee JH, Kim DH, Song SH| title=Fibroepithelial polyp of the vulva accompanied by lymphangioma circumscriptum. | journal=Obstet Gynecol Sci | year= 2017 | volume= 60 | issue= 4 | pages= 401-404 | pmid=28791276 | doi=10.5468/ogs.2017.60.4.401 | pmc=5547092 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28791276  }} </ref><ref name="pmid28693464">{{cite journal| author=Ten Donkelaar CS, Houwert AC, Ten Kate FJW, Lock MTWT| title=Polypoid arteriovenous malformation of the ureter mimicking a fibroepithelial polyp, a case report. | journal=BMC Urol | year= 2017 | volume= 17 | issue= 1 | pages= 55 | pmid=28693464 | doi=10.1186/s12894-017-0237-z | pmc=5504856 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28693464  }} </ref><ref name="pmid28603622">{{cite journal| author=Saito N, Yamasaki M, Daido W, Ishiyama S, Deguchi N, Taniwaki M| title=A bronchial fibroepithelial polyp with abnormal findings on auto-fluorescence imaging. | journal=Respirol Case Rep | year= 2017 | volume= 5 | issue= 5 | pages= e00244 | pmid=28603622 | doi=10.1002/rcr2.244 | pmc=5465754 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28603622  }} </ref>
|
|Associated with:
|
* [[Human papillomavirus|HPV 6]] (low risk)
|
* [[Human papillomavirus|HPV 11]]
|
|
* Fibrovascular [[Core (anatomy)|cores]] covered by [[squamous epithelium]]
* Larger [[lesions]] may have a [[Flat affect|flattened]] [[Epidermis (skin)|epidermis]]
* Smaller [[lesions]] can have [[epidermal]] [[hyperplasia]] or [[seborrheic keratosis]]-like changes
* [[Central]] [[Core (anatomy)|core]] composed of [[Loose connective tissue|loose]] [[collagen]] with increased [[blood vessels]]
* In larger [[lesion]], may have a [[central]] [[Core (anatomy)|core]] of [[adipose tissue]]
* [[Pagetoid]] [[Dyskeratosis congenita|dyskeratosis]] is sometimes present as an [[incidental finding]]
* May have [[ischemic necrosis]] due to [[torsion]]
|Positive for:
* [[Desmin]]
* [[Vimentin]]
* [[ER]]
* [[PR]]
Negative for:
* [[Actin]]
|Associated with:
* [[Diabetes]] (elevated [[blood sugar]] and [[insulin]])
* [[Abnormal]] [[lipid profile]]
* Other components of [[metabolic syndrome]]
* [[Birt-Hogg-Dubé syndrome|Birt-Hogg-Dube syndrome]]
* [[Acromegaly]]
* [[Polycystic ovary syndrome]]
* May increase in [[number]] during [[pregnancy]]
|
|
* Occurs usually in intertriginous [[Area|areas]] (i.e. [[axilla]], [[groin]])
* [[Face]]
* [[Neck]]
* [[Eyelids]]
* [[Vulva]]
* [[Tonsils]]
* [[Ureter]]
* [[Bowel]]
* [[Urinary bladder]]
* [[Bronchi]]
|
|
* [[Soft tissue|Soft]] [[papilloma]], [[flesh]] [[Color|colored]] to dark [[brown]], [[sessile]] to [[pedunculated]]
* A few [[millimeters]] to multiple [[Centimeter|centimeters]] in size
* Larger [[lesions]] often [[Attachment theory|attach]] to [[skin]] by slender stalks
|
|
* [[Benign]] [[skin]] [[lesions]] in [[Adult|adults]], excised for cosmetic [[Reasoning|reasons]]
Also known as:
* [[Skin tags|Skin tag]]
* [[Soft tissue|Soft]] [[fibroma]]
* [[Cutaneous]] [[papilloma]]
* [[Cutaneous]] tag
* [[Fibroma]] pendulum
* [[Fibroma]] [[molluscum]]
|}
|}



Latest revision as of 16:12, 1 November 2019

Neurofibroma Microchapters

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Overview

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Differentiating Neurofibroma from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

Neurofibroma must be differentiated from schwannoma, dermatofibrosarcoma protuberans (DFSP), ganglioneuroma, dermal neurotized melanocytic nevus, myxoid liposarcoma, solitary circumscribed neuroma, traumatic neuroma, superficial angiomyxoma, nerve sheath myxoma, malignant peripheral nerve sheath tumor, spindle cell lipoma, leiomyoma, inflammatory myofibroblastic tumor, and fibroepithelial polyp.

Differential Diagnosis

Neurofibroma must be differentiated from:

Differentiating neurofibroma from other diseases
Disease entity Etiology (Genetic or others) Histopathological findings Immunohistochemical staining Risk factors Common site of involvement Clinical manifestations Other associated features
Neurofibroma[1][2][3][4][5][6][7][4][8][9][10][11]

Can be sporadic or as a part of Neurofibromatosis 1 and 2

Neurofibroma with degenerative atypia ("ancient change") has following microscopic features:

Positive for:

Negative for:

Schwannoma[12][13][14][15][16] Positive for:

Negative for:

Symptoms of schwannoma depend on the location of the tumor:

Palisaded encapsulated neuroma (PEN) /solitary circumscribed neuroma[17] Positive for:

Negative for:

90% lesions affect the face involving:

Remaining 10% can occur anywhere in body involving:

Traumatic neuroma[18][19][20][21] Positive for: Most common oral locations are:

Rarely involves:

Also known as:
Neurotized melanocytic nevus[22][23][24][25] Positive for:

Negative for:

Can occur anywhere in body, mostly involving following areas: _
Cutaneous myxoma (Superficial angiomyxoma)[26][27][28][29] Positive for:

Negative for:

Associated with Carney's complex/syndrome which includes following:

May be associated with NAME or LAMB syndrome

Nerve sheath myxoma[30][31][32][33][34][35] Positive for: _ Can occur anywhere in body:
Malignant peripheral nerve sheath tumor (MPNST)/malignant schwannoma[36][37][38][39][40][41]

Electron microscopy shows:

Positive for:

In case of glandular differentiation (malignant), positive for:

Negative for:

Associated with:

May be associated with:

Bulky deep-seated tumor usually arising from major nerves in:
Dermatofibrosarcoma protuberans (DFSP) Positive for:

Negative for:

_
Spindle cell lipoma Positive for:

Spindle cells are negative for:

_
  • Multiple well-circumscribed painless nodules involving several body parts
_
Ganglioneuroma[42][43] Genes involved in the pathogenesis of ganglioneuroma include:

Two histologic subtypes:

Positive for:

Negative for:

Ganglioneuromas may be associated with:

Located along distribution of sympathetic nervous system:

Symptoms of ganglioneuroma vary depending on the location of tumor, and include the following:

Patients with ganglioneuroma may also have paraneoplastic syndrome, which may manifest with:

Ganglioneuromas are included in the neuroblastic tumors group, which includes:

Myxoid liposarcoma[44][45][46][47][48][49][50][51][52][53]

Atypical lipomatous tumor/well differentiated liposarcoma and dedifferentiated liposarcoma are associated with:

Myxoid liposarcoma is associated with:

Pleomorphicliposarcoma is associated with:

Well-differentiated liposarcoma:

De-differentiated liposarcoma:

Atypical lipomatous tumor/well differentiated liposarcoma is positive for:

_
Leiomyoma[54][55][56][57][58][59][60][61][55][58][62]

Positive for:

Negative for:

_
Inflammatory myofibroblastic tumor(IMT)[54][55][56][57][58][59][60][61][55][58][62]

Unknown underlying etiology, may be due to inflammatory reaction to:

Mutations such as:

Positive for:

Negative for:

Also known as:

Fibroepithelial polyp/Acrochordon[63][64][65][66][67][68][69][70][71][72][73][74][75][76][77][78][79][80][81][82] Associated with: Positive for:

Negative for:

Associated with:

Also known as:

References

  1. Rodriguez, Fausto J.; Folpe, Andrew L.; Giannini, Caterina; Perry, Arie (2012). "Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems". Acta Neuropathologica. 123 (3): 295–319. doi:10.1007/s00401-012-0954-z. ISSN 0001-6322.
  2. Choi, Kwangmin; Komurov, Kakajan; Fletcher, Jonathan S.; Jousma, Edwin; Cancelas, Jose A.; Wu, Jianqiang; Ratner, Nancy (2017). "An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system". Scientific Reports. 7 (1). doi:10.1038/srep43315. ISSN 2045-2322.
  3. Liao, Chung-Ping; Booker, Reid C.; Brosseau, Jean-Philippe; Chen, Zhiguo; Mo, Juan; Tchegnon, Edem; Wang, Yong; Clapp, D. Wade; Le, Lu Q. (2018). "Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis". Journal of Clinical Investigation. 128 (7): 2848–2861. doi:10.1172/JCI99424. ISSN 0021-9738.
  4. 4.0 4.1 Staser, K.; Yang, F.-C.; Clapp, D. W. (2010). "Mast cells and the neurofibroma microenvironment". Blood. 116 (2): 157–164. doi:10.1182/blood-2009-09-242875. ISSN 0006-4971.
  5. Muir, David; Neubauer, Debbie; Lim, Ingrid T.; Yachnis, Anthony T.; Wallace, Margaret R. (2001). "Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells". The American Journal of Pathology. 158 (2): 501–513. doi:10.1016/S0002-9440(10)63992-2. ISSN 0002-9440.
  6. Wilkinson, Lana M.; Manson, David; Smith, Charles R. (2004). "Best Cases from the AFIP". RadioGraphics. 24 (suppl_1): S237–S242. doi:10.1148/rg.24si035170. ISSN 0271-5333.
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  8. Mautner, V. F.; Friedrich, R. E.; von Deimling, A.; Hagel, C.; Korf, B.; Knöfel, M. T.; Wenzel, R.; Fünsterer, C. (2003). "Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma". Neuroradiology. 45 (9): 618–625. doi:10.1007/s00234-003-0964-6. ISSN 0028-3940.
  9. Shen, M H; Harper, P S; Upadhyaya, M (1996). "Molecular genetics of neurofibromatosis type 1 (NF1)". Journal of Medical Genetics. 33 (1): 2–17. doi:10.1136/jmg.33.1.2. ISSN 1468-6244.
  10. Rubin, Joshua B.; Gutmann, David H. (2005). "Neurofibromatosis type 1 — a model for nervous system tumour formation?". Nature Reviews Cancer. 5 (7): 557–564. doi:10.1038/nrc1653. ISSN 1474-175X.
  11. Gray, Mark H. (1990). "Immunohistochemical Demonstration of Factor XIIIa Expression in Neurofibromas". Archives of Dermatology. 126 (4): 472. doi:10.1001/archderm.1990.01670280056009. ISSN 0003-987X.
  12. Schwannoma. Dr Tim Luijkx and Dr Sara Wein et al. http://radiopaedia.org/articles/schwannoma
  13. Vestibular Schwannoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Vestibular_schwannoma Accessed on October 2 2015
  14. Giordano J, Rogers LV (1989). "Peripherally administered serotonin 5-HT3 receptor antagonists reduce inflammatory pain in rats". European Journal of Pharmacology. 170 (1–2): 83–6. PMID 2612565. |access-date= requires |url= (help)
  15. Kolvenbach H, Lauven PM, Schneider B, Kunath U (1989). "Repetitive intercostal nerve block via catheter for postoperative pain relief after thoracotomy". The Thoracic and Cardiovascular Surgeon. 37 (5): 273–6. doi:10.1055/s-2007-1020331. PMID 2588243. Retrieved 2015-11-20.
  16. Opaleva-Stegantseva VA, Ivanov AG, Gavrilina IA, Khar'kov EI, Ratovskaia VI (1986). "[Incidence of sudden death cases in acute coronary insufficiency and acute myocardial infarction at the pre-hospital stage in Krasnoyarsk]". Kardiologiia (in Russian). 26 (5): 23–6. PMID 3735913. |access-date= requires |url= (help)
  17. Misago N, Inoue T, Narisawa Y (2007). "Unusual benign myxoid nerve sheath lesion: myxoid palisaded encapsulated neuroma (PEN) or nerve sheath myxoma with PEN/PEN-like features?". Am J Dermatopathol. 29 (2): 160–4. doi:10.1097/01.dad.0000256688.91974.09. PMID 17414438.
  18. Lee EJ, Calcaterra TC, Zuckerbraun L (1998). "Traumatic neuromas of the head and neck". Ear Nose Throat J. 77 (8): 670–4, 676. PMID 9745184.
  19. Hanna SA, Catapano J, Borschel GH (2016). "Painful pediatric traumatic neuroma: surgical management and clinical outcomes". Childs Nerv Syst. 32 (7): 1191–4. doi:10.1007/s00381-016-3109-z. PMID 27179535.
  20. Foltán R, Klíma K, Spacková J, Sedý J (2008). "Mechanism of traumatic neuroma development". Med Hypotheses. 71 (4): 572–6. doi:10.1016/j.mehy.2008.05.010. PMID 18599222.
  21. Yao C, Zhou X, Zhao B, Sun C, Poonit K, Yan H (2017). "Treatments of traumatic neuropathic pain: a systematic review". Oncotarget. 8 (34): 57670–57679. doi:10.18632/oncotarget.16917. PMC 5593675. PMID 28915703.
  22. Gray MH, Smoller BR, McNutt NS, Hsu A (1990). "Neurofibromas and neurotized melanocytic nevi are immunohistochemically distinct neoplasms". Am J Dermatopathol. 12 (3): 234–41. PMID 1693815.
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  24. Singh N, Chandrashekar L, Kar R, Sylvia MT, Thappa DM (2015). "Neurotized congenital melanocytic nevus resembling a pigmented neurofibroma". Indian J Dermatol. 60 (1): 46–50. doi:10.4103/0019-5154.147789. PMC 4318062. PMID 25657396.
  25. Gray MH, Smoller BR, McNutt NS, Hsu A (1990). "Immunohistochemical demonstration of factor XIIIa expression in neurofibromas. A practical means of differentiating these tumors from neurotized melanocytic nevi and schwannomas". Arch Dermatol. 126 (4): 472–6. PMID 1690969.
  26. https://www.sciencedirect.com/topics/medicine-and-dentistry/cutaneous-myxoma
  27. Alaiti, Samer; Nelson, Fern P.; Ryoo, Jei W. (2000). "Solitary cutaneous myxoma". Journal of the American Academy of Dermatology. 43 (2): 377–379. doi:10.1067/mjd.2000.101878. ISSN 0190-9622.
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