Liver and intrahepatic bile ducts - tumor
Other malignancies
Epithelioid hemangioendothelioma

Topic Completed: 1 February 2012

Revised: 24 April 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Epithelioid hemangioendothelioma[TI] liver[TI]

Deepali Jain, M.D.
Page views in 2018: 8,446
Page views in 2019 to date: 7,152
Cite this page: Jain D. Epithelioid hemangioendothelioma. website. Accessed October 18th, 2019.
Definition / general
  • Endothelial derived neoplasm with unpredictable clinical course, usually intermediate between hemangioma and angiosarcoma
  • Often misdiagnosed
Clinical features
  • Mean age 47 years but occurs at any age, 60% women
  • No predisposing factors; occasional cases reported in vinyl chloride workers, women on oral contraceptives, hepatitis B / C patients, following hepatic trauma
  • Symptoms: abdominal pain, weight loss, hepatic venous outflow obstruction; 40% are asymptomatic
  • FNA not recommended as even small biopsies can be misleading
  • Indolent and slow growing; 5 year survival of 43%
  • 50% have extrahepatic involvement at diagnosis, which does not preclude long survival
  • Metastases to abdominal lymph nodes, lung, omentum or peritoneum, spleen
  • Radiologic calcifications in 20%; peripheral nodules with capsular retraction by CT scan
Prognostic factors
  • High cellularity is unfavorable but histology is otherwise not prognostic
Case reports
  • Resection, liver transplantation
Gross description
  • Multiple (80%), tan gray, firm, circumscribed and focally confluent nodules up to 12 cm with infiltrative borders
  • May involve venous structures as intravascular proliferations or fibrothrombotic occlusions
  • Remaining liver is unremarkable
Gross images

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Mottled tumor with cavernous hemangioma

Various images

Microscopic (histologic) description
  • Zonal pattern; periphery shows sinusoidal proliferation with tufting of tumor cells within portal vein branches
  • Midzone has sinusoidal obliteration with atrophic hepatocyte plates and increased myxochondroid and sclerotic stroma
  • Perivenular stroma is paucicellular and often calcified
  • Epithelioid and fibromyxoid tumor cells have focal intracytoplasmic vacuoles containing red blood cells embedded in fibromyxoid matrix
  • Epithelioid cells are rounded with eosinophilic cytoplasm, mild to moderately atypical nuclei with prominent nucleoli and no / rare mitotic figures
  • Extramedullary hematopoiesis present; may have myxoid areas; often has infiltrative margins, inflammatory infiltrate; adjacent liver usually normal
Microscopic (histologic) images

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Case of the Week #341:





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Various images

Microvascular channels and intracytoplasmic vacuoles

Intracytoplasmic vacuoles and projection into vein

Cytology images

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Various images

Positive stains
Negative stains
Electron microscopy description
  • Weibel-Palade bodies, intermediate filaments
Molecular / cytogenetics description
  • t(1;3)(p36.3;q25) has been reported
Differential diagnosis
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