Epithelioid hemangioendothelioma of the liver in primary biliary cirrhosis. A case report.

We report a case of primary epithelioid hemangioendothelioma of the liver occurring in a patient with primary biliary cirrhosis, stage III. The hepatic tumor was found incidentally by imaging techniques and was surgically resected under a tentative diagnosis of metastatic carcinoma. The resected tumor (1.8 x 1.6 cm) showed typical histologic features of epithelioid hemangioendothelioma. The tumor cells were positive for factor VIII-related antigen and were stained with Ulex europaeus lectin I. Ultrastructurally, the tumor cells showed cytoplasmic vacuoles, tight junctions, basal lamina, pinocytotic vesicles, bundles of thin filaments (approximately 10 nm in diameter) and Weibel-Palade bodies. The non-tumorous part of the liver showed features of primary biliary cirrhosis, stage III. This is the first reported case of epithelioid hemangioendothelioma occurring in a liver with primary biliary cirrhosis.