Mesenchymal tumors

Epithelioid hemangioendothelioma

Last author update: 1 September 2012
Last staff update: 1 October 2020

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PubMed Search: pulmonary epithelioid hemangioendothelioma

Deepali Jain, M.D.
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Cite this page: Jain D. Epithelioid hemangioendothelioma. website. Accessed September 21st, 2023.
Definition / general
  • Formerly called intravascular bronchioloalveolar tumor (see also Soft tissue topic)
  • 80% women, usually young adults
  • Neoplastic, but usually not metastatic
  • Progressive growth, usually remains within thoracic cavity, may cause death from respiratory insufficiency
  • Other sites: liver, bone
  • 10% have peripheral eosinophilia
  • Poor prognosis if vascular spread, pleural involvement, severe symptoms
Case reports
Gross description
  • Multiple round, well demarcated nodules < 2 cm, often in lower lung, with a gray-white peripheral rim and chondroid appearance
  • Resembles metastasis, granulomatous disease or interstitial lung disease
  • May spread along pleura or pericardium and resemble mesothelioma
Microscopic (histologic) description
  • Central hyalinized stroma, eosinophilic amorphous material or coagulative necrosis with variable calcification surrounded by thin rim of plump eosinophilic endothelial cells
  • Clusters fill alveoli, apparently through pores of Kohn, and occasionally bronchioles, arteries, veins
  • Nuclei are bland, round / oval, may have cytoplasmic vacuoles
  • No / minimal mitoses
  • Lung architecture preserved
Positive stains
Electron microscopy description
  • Endothelial features: well developed basal lamina, pinocytotic vesicles, occasional Weibel-Palade bodies
Differential diagnosis
  • Metastatic tumor from liver or other sites: destroy lung architecture, high mitotic rate
  • Sclerosing hemangioma: destroys lung architecture, negative for vascular markers
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