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Difference between revisions of "Epithelioid hemangioendothelioma"
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Epithelioid hemangioendothelioma (view source)
Revision as of 14:31, 5 April 2024
, 14:31, 5 April 2024no edit summary
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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 = | ||
| Grossing = | | Grossing = | ||
| Site = [[soft tissue lesions|soft tissue]] - see ''[[vascular tumours]]'' | | Site = [[soft tissue lesions|soft tissue]] - see ''[[vascular tumours]]'', classically [[liver]] - but various sites reported | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
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==Gross== | ==Gross== | ||
* | *Classically, a [[liver]] lesion - but found elsewhere.<ref>{{Cite journal | last1 = Cardinal | first1 = J. | last2 = de Vera | first2 = ME. | last3 = Marsh | first3 = JW. | last4 = Steel | first4 = JL. | last5 = Geller | first5 = DA. | last6 = Fontes | first6 = P. | last7 = Nalesnik | first7 = M. | last8 = Gamblin | first8 = TC. | title = Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases. | journal = Arch Surg | volume = 144 | issue = 11 | pages = 1035-9 | month = Nov | year = 2009 | doi = 10.1001/archsurg.2009.121 | PMID = 19917940 }}</ref><ref name=pmid37541086>{{cite journal |authors=Haughey AM, Moloney BM, O'Brien CM |title=Epithelioid Haemangioendothelioma; Not simply a hepatic pathology |journal=Clin Imaging |volume=102 |issue= |pages=42–52 |date=October 2023 |pmid=37541086 |doi=10.1016/j.clinimag.2023.07.003 |url=}}</ref> | ||
*Case reports of EHE in a wide number of anatomical sites (bowel,<ref name=pmid30238810>{{cite journal |authors=Spasic S, Brcic I, Freire R, Garcia-Buitrago MT, Rosenberg AE |title=Epithelioid Hemangioendothelioma of the Bowel in Crohn's Disease: The First Reported Case |journal=Int J Surg Pathol |volume=27 |issue=4 |pages=423–426 |date=June 2019 |pmid=30238810 |doi=10.1177/1066896918801527 |url=}}</ref>, parotid<ref name=pmid31530411>{{cite journal |authors=Suarez-Zamora DA, Rodriguez-Urrego PA, Hakim-Tawil JA, Palau-Lazaro MA |title=Epithelioid hemangioendothelioma of the parotid gland: A case report in an unusual location with a review of the literature |journal=Rev Esp Patol |volume=52 |issue=4 |pages=260–264 |date=2019 |pmid=31530411 |doi=10.1016/j.patol.2019.04.002 |url=}}</ref> mediastinum<ref>{{cite journal |authors=Kim SH, Kim YS, Jang MH, Kwon HJ |title=Mediastinal Epithelioid Hemangioendothelioma Invading Superior Vena Cava: A Case Report and Review of Literature |journal=Curr Med Imaging Rev |volume=15 |issue=3 |pages=349–352 |date=2019 |pmid=31989887 |doi=10.2174/1573405614666180124141817 |url=}}</ref>). | |||
==Microscopic== | ==Microscopic== | ||
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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== | |||
Gene fusions:<ref name=pmid26414223/> | |||
*WWTR1-CAMTA1 - seen in approximately 90% of cases. | |||
*YAP1-TFE3 fusion gene - <5% of cases. | |||
==See also== | ==See also== | ||
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*[[Kaposiform hemangioendothelioma]]. | *[[Kaposiform hemangioendothelioma]]. | ||
*[[Hemangioendothelioma]]. | *[[Hemangioendothelioma]]. | ||
*[[Liver hemangioma]]. | |||
==References== | ==References== | ||