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Epithelioid hemangioendothelioma of the liver: A rare hepatic tumor
Author(s) -
Läuffer Jörg M.,
Zimmermann Arthur,
Krähenbühl Lukas,
Triller Jürgen,
Baer Hans U.
Publication year - 1996
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/(sici)1097-0142(19961201)78:11<2318::aid-cncr8>3.0.co;2-i
Subject(s) - medicine , epithelioid hemangioendothelioma , hemangioendothelioma , pathology , liver transplantation , metastasis , hemangioma , soft tissue , neoplasm , transplantation , immunohistochemistry , surgery , cancer
BACKGROUND Epithelioid hemangioendothelioma (EH) is a rare neoplasm of vascular origin that may develop at different sites, such as in soft tissue, the lungs, or the liver. It usually affects adult females, and its unpredictable malignant potential has a range between benign hemangioma and clearly malignant hemangioendotheliosarcoma. METHODS In the current study, the authors describe 2 patients with primary EH of the liver and review 127 previously published cases found in the literature. RESULTS Most patients presented with nonspecific symptoms, such as right upper quadrant abdominal pain or weight loss. The tumors usually presented as multiple nodular lesions involving both lobes of the liver. Overall metastasis rate was 45.1%, with preferential involvement of the lungs and bones. In general, the key to diagnosis was the demonstration of cells containing factor VIII‐related antigen. CONCLUSIONS EH of the liver is a very rare clinical entity. The primary treatments of choice are radical hepatic resection or orthotopic liver transplantation. The 5‐year survival of 55.5% is significantly better than for other hepatic malignancies. Cancer 1996;78:2318‐27.