Difference between revisions of "Epithelioid hemangioendothelioma"
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*Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/> | *Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/> | ||
*Rare - no standardized treatment.<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> | *Rare - no standardized treatment.<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> | ||
==Gross== | |||
*Relatively often in the [[liver]].<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> | |||
==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]]. | |||
===Images=== | ===Images=== | ||
Revision as of 00:34, 3 April 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 |
Epithelioid hemangioendothelioma, abbreviated EHE, is rare malignant vascular tumour.
It should not be confused with epithelioid hemangioma.
General
- Malignant.[1]
- Adults - wide age range.
- Associated with oral contraceptives, vinyl chloride.[2]
- Rare - no standardized treatment.[3]
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:[5]
- Angiosarcoma, epithelioid.
- Hemangioma.
- Cholangiocarcinoma.
- Fibrolamellar hepatocellular carcinoma.
Images
Epithelioid hemangioendothelioma. (WC)
www:
- Epithelioid hemangioendothelioma - low mag. (flickr.com/Rosen).
- Epithelioid hemangioendothelioma - high mag. (flickr.com/Rosen).
- Epithelioid hemangioendothelioma (surgicalpathologyatlas.com).
IHC
Features:[2]
- CD31 +ve.
- CD34 +ve.
- Factor VIII +ve.
See also
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.