Hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origi n with unpredictable malignant potential. We describe two patients with pri mary EHE of the liver and review 34 cases previously published in Japan and compare them with those in Western countries. The clinical aspects of EHE and relevant treatment results in Japanese patients were similar to those f ound in Western countries, the exception being the matter of difference in incidence between genders. Although this tumor type has been reported to be more frequent among women, there was no significant difference in incidenc e between men and women. Hepatic EHE generally behaves as a low-grade malig nant tumor with a slow progression phenotype; however, this disease seems t o be resistant to chemotherapy and to be lethal in some cases. Surgical res ection or liver transplantation is recommended after rapid diagnosis by rad iologic examination and histologic findings, including positive staining of tumor cells for factor VIII-related antigen.