Difference between revisions of "Epithelioid hemangioendothelioma"

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*Excision<ref name=pmid8941001/> if feasible.
*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>
*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:
Prognosis - liver:

Revision as of 17:53, 27 January 2015

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:

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.

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.