Soft tissue
Low / intermediate grade vascular tumors
Epithelioid / histiocytic hemangioendothelioma

Author: Vijay Shankar, M.D.

Revised: 31 January 2018, last major update November 2012

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

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

Related topics: Hemangioendothelioma

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Cite this page: Shankar, V. Epithelioid / histiocytic hemangioendothelioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuehemangioendotheliomaepithelioid.html. Accessed June 24th, 2019.
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
Treatment
  • Low grade tumors: wide local excision
  • High grade tumors: radical local excision with possible neck dissection (head & neck tumors)
Clinical images
Images hosted on PathOut server:

Courtesy of Dr. Mark R. Wick

Great toe, courtesy of Dr. Mark R. Wick



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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
Images hosted on PathOut server:

Breast, courtesy of Dr. Mark R. Wick

Anaplastic variant, courtesy of Dr. Mark R. Wick

CD141, courtesy of Dr. Mark R. Wick

von Willebrand factor, courtesy of Dr. Mark R. Wick


Courtesy of Dr. Mark R. Wick

Courtesy of Dr. Mark R. Wick



Case of the Week #77:

Thigh tumor - H&E and CD31



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Forearm mass

Spine

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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