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      =
Line 67: Line 67:
*[[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==

Revision as of 13:25, 4 April 2024

Epithelioid hemangioendothelioma
Diagnosis in short

Epithelioid hemangioendothelioma. H&E stain.

LM 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
LM DDx epithelioid angiosarcoma, hemangioma, epithelioid sarcoma
IHC CD31 +ve, CD34 +ve, factor VIII +ve, CAMTA1 +ve, TFE3 +ve/-ve
Molecular gene fusions: WWTR1-CAMTA1 (approximately 90% of cases), YAP1-TFE3 (small number of cases)
Site soft tissue - see vascular tumours

Prevalence rare
Prognosis moderate
Treatment resection

Epithelioid hemangioendothelioma, abbreviated EHE, is rare malignant vascular tumour.

It should not be confused with epithelioid hemangioma.

General

Treatment:

Prognosis - liver:

Gross

Microscopic

Features:[2]

  • Large epithelioid perivascular cells with:
    • Abundant pale eosinophilic cytoplasm.
    • Cytoplasmic vacuolation (some cells) - AKA "blister cells" - key feature.
      • May form lumen and have RBC within.
    • Vesicular nucleus with prominent nucleolus in some cells.
  • Tuft-like projections into capillaries.
  • Tumour cells may be in well-circumscribed paucicellular nodules or more cellular poorly formed aggregates.

DDx:[7]

Images

www:

IHC

Features:[2]

  • CD31 +ve.
  • CD34 +ve.
  • Factor VIII +ve.
  • CAMTA1 +ve.[8]
  • TFE3 +ve - minority of cases.

Molecular

Fusions:[8]

  • WWTR1-CAMTA1 - seen in approximately 90% of cases.
  • YAP1-TFE3 fusion gene - <5% of cases.

See also

References

  1. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 603. ISBN 978-0781765275.
  2. 2.0 2.1 2.2 Gupta, R.; Mathur, SR.; Gupta, SD.; Durgapal, P.; Iyer, VK.; Das, CJ.; Shalimar, SK.; Acharya, . (2010). "Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology.". Cytojournal 6: 25. doi:10.4103/1742-6413.58951. PMID 20165548.
  3. 3.0 3.1 Chevreau, C.; Le Cesne, A.; Ray-Coquard, I.; Italiano, A.; Cioffi, A.; Isambert, N.; Robin, YM.; Fournier, C. et al. (Jul 2013). "Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO).". Cancer 119 (14): 2639-44. doi:10.1002/cncr.28109. PMID 23589078.
  4. 4.0 4.1 Läuffer, JM.; Zimmermann, A.; Krähenbühl, L.; Triller, J.; Baer, HU. (Dec 1996). "Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor.". Cancer 78 (11): 2318-27. PMID 8941001.
  5. Nudo, CG.; Yoshida, EM.; Bain, VG.; Marleau, D.; Wong, P.; Marotta, PJ.; Renner, E.; Watt, KD. et al. (Oct 2008). "Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience.". Can J Gastroenterol 22 (10): 821-4. PMID 18925305.
  6. Cardinal, J.; de Vera, ME.; Marsh, JW.; Steel, JL.; Geller, DA.; Fontes, P.; Nalesnik, M.; Gamblin, TC. (Nov 2009). "Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.". Arch Surg 144 (11): 1035-9. doi:10.1001/archsurg.2009.121. PMID 19917940.
  7. Cardinal, J.; de Vera, ME.; Marsh, JW.; Steel, JL.; Geller, DA.; Fontes, P.; Nalesnik, M.; Gamblin, TC. (Nov 2009). "Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.". Arch Surg 144 (11): 1035-9. doi:10.1001/archsurg.2009.121. PMID 19917940.
  8. 8.0 8.1 8.2 Doyle LA, Fletcher CD, Hornick JL (January 2016). "Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics". Am J Surg Pathol 40 (1): 94–102. doi:10.1097/PAS.0000000000000511. PMID 26414223.