Epithelioid hemangioendothelioma of the liver: A rare hepatic tumor

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.