Lymph nodes & spleen, nonlymphoid
Lymph nodes-vascular lesions
Epithelioid hemangioendothelioma

Topic Completed: 1 April 2014

Minor changes: 31 July 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Epithelioid hemangioendothelioma[TI] free full text[sb]

Patricia Tsang, M.D., M.B.A.
Page views in 2019: 1,322
Page views in 2020 to date: 588
Cite this page: Tsang P. Epithelioid hemangioendothelioma. website. Accessed August 12th, 2020.
Definition / general
  • Very rare vascular neoplasm with borderline biological behavior and intermediate malignant potential
  • Most cases are nodal metastases from skin, soft tissue or visceral organs
Clinical features
  • Intermediate in clinical course between (benign) hemangioma and (malignant) angiosarcoma
  • More common in adults than children
Case reports
  • Excision
  • May recur locally or metastasize
Microscopic (histologic) description
  • Cords of polygonal or plump spindle cells in a myxohyaline or chondroid matrix
  • Often central necrosis and hyalinization
  • Tumor cells have abundant eosinophilic hyaline cytoplasm with intracytoplasmic vacuoles that may contain red blood cells (reminiscent of primitive vascular channels)
  • Vesicular nuclei with mild / moderate pleomorphism and nuclear pseudoinclusions; inconspicuous nucleoli
  • Rare mitotic figures
  • Can mimic metastatic carcinoma due to epithelioid cytology, intracytoplasmic vacuoles and variable cytokeratin expression
Microscopic (histologic) images

AFIP images

Lymph node tumors: H&E and factor VIII

With regressive changes

Polymorphous hemangioendothelioma

Negative stains
Molecular / cytogenetics description
Spindle cell variant
Definition / general
  • Rare vascular lesion with endothelial cell and smooth muscle cell proliferation
  • Predominates in dermis and subcutaneous tissue of extremities; primary lymph node involvement is very uncommon

  • Postulated vascular malformation (nonneoplastic), characterized by alternating areas of cavernous blood spaces and cellular areas with spindle cells and epithelioid cells
  • Endothelial cell intracytoplasmic vacuoles represent primitive vascular differentiation

Clinical features
  • May recur locally after incomplete excision
  • No known metastatic potential
  • Excellent prognosis

Case reports
Microscopic (histologic) description
  • Prominent spindle cell component in solid areas containing slit-like vascular spaces
  • Cavernous vascular spaces, some may be lined by plump epithelioid endothelial cells with intracytoplasmic vacuoles
  • Organizing intravascular thrombi are common
  • Low mitotic rate, no obvious pleomorphism

Microscopic (histologic) images

Images hosted on other servers:

Spindle cell hemangioma

Positive stains
Negative stains
  • Spindle cells: CD34-

Differential diagnosis
Additional references
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