Soft tissue
Intermediate (locally aggressive / rarely metastasizing)
Epithelioid hemangioendothelioma

Topic Completed: 1 November 2012

Minor changes: 4 November 2020

Copyright: 2002-2020,, Inc.

PubMed search: Epithelioid [title] OR histiocytic [title] hemangioendothelioma soft tissue

Related topics: Hemangioendothelioma

Vijay Shankar, M.D.
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Page views in 2020 to date: 14,131
Cite this page: Shankar V. Epithelioid hemangioendothelioma. website. Accessed December 5th, 2020.
Definition / general
  • Intermediate grade vascular malignancies closely associated with or arising from a vein in 50% of cases
  • Usually adults, 60% women
  • Extremities (60%); also head and neck, mediastinum, trunk
  • Unpredictable clinical course, but less aggressive than angiosarcoma
  • 13% recur, 20 - 30% metastasize (lung, lymph nodes), 13% die of disease (Am J Surg Pathol 1997;21:363); for lung, mortality is 65%
  • High risk (> 3 MF / 50 HPF and size > 3 cm) have 5 year disease specific survival of 59% versus 100% for low risk (Am J Surg Pathol 2008;32:924)
Case reports
  • Low grade tumors: wide local excision
  • High grade tumors: radical local excision with possible neck dissection (head & neck tumors)
Clinical images

Contributed by Dr. Mark R. Wick

Great toe

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Gross description
  • Variable size, up to 18 cm
Microscopic (histologic) description
  • Cords or small nests of round endothelial cells with abundant eosinophilic cytoplasm
  • Tumors arising from vessels extend outward from the lumen towards soft tissue
  • Tumor cells often have intracytoplasmic vacuoles representing small vascular lumina, which may resemble mucin
  • Nuclei are round and may be indented
  • Usually minimal mitotic activity, atypia or necrosis, but 25% of cases exhibit frank malignant features of prominent nuclear pleomorphism, mitotic activity, focal spindling or necrosis
  • Stroma may be scanty or myxoid
  • May have peripheral inflammatory infiltrate with germinal centers and eosinophils, multinucleated giant cells
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick


Anaplastic variant


von Willebrand factor

Case #77

Thigh tumor - H&E and CD31

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Forearm mass


Lower eyelid

Sphenoid bone

Cytology description
Cytology images

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Polygonal cells with moderate atypia

Positive stains
Molecular / cytogenetics description
Differential diagnosis
  • Epithelioid angiosarcoma: irregular sinusoidal vascular channels, solid sheets of cells with marked atypia and prominent mitotic activity, necrosis
  • Epithelioid sarcoma: distal extremities of young adults, tumor cells merge with collagenous stroma, keratin+ (strong), CD31-
  • Melanoma: S100+, HMB45+, CD31-
  • Metastatic carcinoma: more marked atypia, mitotic activity, usually not angiocentric, keratin+, CD31-
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