Difference between revisions of "Epithelioid hemangioendothelioma"

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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/>
*Rare.<ref name=pmid23589078/>


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Prognosis - liver:
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>
*~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.
**Better than other [[liver tumours]].


==Gross==
==Gross==

Revision as of 02:46, 20 September 2014

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
IHC CD31 +ve, CD34 +ve, factor VIII +ve
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:

  • Excision[4] if feasible.
  • Chemotherapy - not standardized.[3]

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:[6]

Images

www:

IHC

Features:[2]

  • CD31 +ve.
  • CD34 +ve.
  • Factor VIII +ve.

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. 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.
  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.