Difference between revisions of "Epithelioid hemangioendothelioma"

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Epithelioid hemangioendothelioma (view source)

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'''Epithelioid hemangioendothelioma''' is rare malignant [[vascular tumours|vascular tumour]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Epithelioid_hemangioendothelioma.jpg
| Width      =
| Caption    = Epithelioid hemangioendothelioma. [[H&E stain]].
| Synonyms  =
| 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  =
| LMDDx      = epithelioid [[angiosarcoma]], [[hemangioma]], [[epithelioid sarcoma]]
| Stains    =
| IHC        = CD31 +ve, CD34 +ve, factor VIII +ve, CAMTA1 +ve, TFE3 +ve/-ve
| EM        =
| Molecular  = gene fusions: WWTR1-CAMTA1 (approximately 90% of cases), YAP1-TFE3 (small number of cases)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - see ''[[vascular tumours]]'', classically [[liver]] - but various sites reported
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = moderate
| Other      =
| ClinDDx    =
| Tx        = resection
}}
'''Epithelioid hemangioendothelioma''', abbreviated '''EHE''', is rare malignant [[vascular tumours|vascular tumour]].


It should '''not''' be confused with ''[[epithelioid hemangioma]]''.
It should '''not''' be confused with ''[[epithelioid hemangioma]]''.
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*Malignant.<ref name=Ref_WMSP603>{{Ref WMSP|603}}</ref>
*Malignant.<ref name=Ref_WMSP603>{{Ref WMSP|603}}</ref>
*Adults - wide age range.
*Adults - wide age range.
*Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/>
*Associated with [[oral contraceptives]], vinyl chloride.<ref name=pmid20165548/>
*Rare.<ref name=pmid23589078/>
 
Treatment:
*Excision<ref name=pmid8941001/> if feasible.
*Chemotherapy - not standardized.<ref name=pmid23589078>{{Cite journal  | last1 = Chevreau | first1 = C. | last2 = Le Cesne | first2 = A. | last3 = Ray-Coquard | first3 = I. | last4 = Italiano | first4 = A. | last5 = Cioffi | first5 = A. | last6 = Isambert | first6 = N. | last7 = Robin | first7 = YM. | last8 = Fournier | first8 = C. | last9 = Clisant | first9 = S. | title = Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO). | journal = Cancer | volume = 119 | issue = 14 | pages = 2639-44 | month = Jul | year = 2013 | doi = 10.1002/cncr.28109 | PMID = 23589078 }}</ref>
*[[Liver transplantation]].<ref>{{Cite journal  | last1 = Nudo | first1 = CG. | last2 = Yoshida | first2 = EM. | last3 = Bain | first3 = VG. | last4 = Marleau | first4 = D. | last5 = Wong | first5 = P. | last6 = Marotta | first6 = PJ. | last7 = Renner | first7 = E. | last8 = Watt | first8 = KD. | last9 = Deschênes | first9 = M. | title = Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience. | journal = Can J Gastroenterol | volume = 22 | issue = 10 | pages = 821-4 | month = Oct | year = 2008 | doi =  | PMID = 18925305 }}</ref>
 
Prognosis - liver:
*~55% five-year survival.<ref name=pmid8941001>{{Cite journal  | last1 = Läuffer | first1 = JM. | last2 = Zimmermann | first2 = A. | last3 = Krähenbühl | first3 = L. | last4 = Triller | first4 = J. | last5 = Baer | first5 = HU. | title = Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor. | journal = Cancer | volume = 78 | issue = 11 | pages = 2318-27 | month = Dec | year = 1996 | doi =  | PMID = 8941001 }}</ref>
**Better than other [[liver tumours]].
 
==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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*Tumour cells may be in well-circumscribed paucicellular nodules ''or'' more cellular poorly formed aggregates.
*Tumour cells may be in well-circumscribed paucicellular nodules ''or'' more cellular poorly formed aggregates.


DDx:
DDx:<ref name=pmid19917940>{{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>
*[[Angiosarcoma]], epithelioid.
*[[Angiosarcoma]], epithelioid.
*[[Hemangioma]].
*[[Hemangioma]].
*[[Cholangiocarcinoma]].
*[[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==
*[[Vascular tumours]].
*[[Vascular tumours]].
*[[Kaposiform hemangioendothelioma]].
*[[Hemangioendothelioma]].
*[[Liver hemangioma]].


==References==
==References==
Michael
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