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Difference between revisions of "Epithelioid hemangioendothelioma"
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| Micro = large epithelioid perivascular cells with abundant pale eosinophilic cytoplasm and cytoplasmic vacuolation ("blister cells") - may form lumen and have RBC within, vesicular nucleus +/-prominent nucleolus; tuft-like projections into capillaries; cells may be in well-circumscribed paucicellular nodules ''or'' poorly formed cellular aggregates | | Micro = large epithelioid perivascular cells with abundant pale eosinophilic cytoplasm and cytoplasmic vacuolation ("blister cells") - may form lumen and have RBC within, vesicular nucleus +/-prominent nucleolus; tuft-like projections into capillaries; cells may be in well-circumscribed paucicellular nodules ''or'' poorly formed cellular aggregates | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = epithelioid [[angiosarcoma]], [[hemangioma]] | | LMDDx = epithelioid [[angiosarcoma]], [[hemangioma]], [[epithelioid sarcoma]] | ||
| Stains = | | Stains = | ||
| IHC = CD31 +ve, CD34 +ve, factor VIII +ve | | IHC = CD31 +ve, CD34 +ve, factor VIII +ve, CAMTA1 +ve, TFE3 +ve/-ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = gene fusions: WWTR1-CAMTA1 (approximately 90% of cases), YAP1-TFE3 (small number of cases) | ||
| IF = | | IF = | ||
| Gross = | | Gross = | ||
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*[[Cholangiocarcinoma]]. | *[[Cholangiocarcinoma]]. | ||
*[[Fibrolamellar hepatocellular carcinoma]]. | *[[Fibrolamellar hepatocellular carcinoma]]. | ||
*[[Epithelioid sarcoma]].<ref name=pmid26414223/> | |||
===Images=== | ===Images=== | ||
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*CD34 +ve. | *CD34 +ve. | ||
*Factor VIII +ve. | *Factor VIII +ve. | ||
*CAMTA1 +ve.<ref name=pmid26414223>{{cite journal |authors=Doyle LA, Fletcher CD, Hornick JL |title=Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics |journal=Am J Surg Pathol |volume=40 |issue=1 |pages=94–102 |date=January 2016 |pmid=26414223 |doi=10.1097/PAS.0000000000000511 |url=}}</ref> | |||
*TFE3 +ve - minority of cases. | |||
==Molecular== | |||
Fusions:<ref name=pmid26414223/> | |||
*WWTR1-CAMTA1 - seen in approximately 90% of cases. | |||
*YAP1-TFE3 fusion gene - <5% of cases. | |||
==See also== | ==See also== | ||