CNS tumor
Mesenchymal and other tumors
Epithelioid hemangioendothelioma

Topic Completed: 1 January 2006

Revised: 3 January 2018, last major update January 2006

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Epithelioid hemangioendothelioma CNS tumor

Nat Pernick, M.D.
Page views in 2018: 567
Page views in 2019 to date: 506
Cite this page: Pernick N. Epithelioid hemangioendothelioma. website. Accessed December 6th, 2019.
Definition / general
  • Rare; usually cerebral hemispheres
  • Intermediate grade between hemangioma (benign) and angiosarcoma (high grade)
  • All ages but 2/3 male
  • Usually unifocal
  • Favorable prognosis with complete resection; may recur or seed neuraxis; deaths are rare
  • Radiologic: associated with peritumoral vasogenic edema and homogenous contrast enhancement
  • References: Am J Surg Pathol 1996;20:707
Case reports
  • Surgical excision, possibly radiation therapy (if incomplete excision) or alpha interferon
Microscopic (histologic) description
  • Histiocytoid, epithelioid and spindled areas with intracytoplasmic lumina
  • Vascular lumina are not apparent or focal
  • Often chronic inflammatory cells, eosinophils and mast cells
  • Variable mitotic figures; no dilated vascular spaces of cavernous hemangioma
  • No significant hemorrhage; no marked atypia
Microscopic (histologic) images

Images hosted on other servers:

H&E, CD34+ and CD31+

Pleura: factor VIII+

Lower extremity tumor

Positive stains
Electron microscopy description
  • Endothelium with Weibel-Palade bodies, abundant intermediate filaments, surrounded by basal lamina
Electron microscopy images

Images hosted on other servers:

Pleura: Weibel-Palade bodies

Molecular / cytogenetics description
  • t(1;3)(p36.3;q25) in some cases (involves PAX7)
Differential diagnosis
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