Hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare, malignant neoplasm of vascular origin arising in soft tissues, lung, and liver. Four cases of hepatic EHE are reported, and 49 previously reported cases are reviewed. The tumor occurs in adults of all ages; 66% of patients are women. The prognosis is variable. Computed tomographic findings suggest the tumor begins as multiple hepatic nodules that grow and coalesce, forming large confluent masses preferentially involving the liver periphery. Extensive hepatic involvement is associated with enlargement of uninvolved portions of the liver and splenomegaly. Awareness of the radiologic features is helpful because clinical findings are nonspecific, and biopsy specimens may be misinterpreted as carcinoma or venoocclusive disease.