Epithelioid hemangioendothelioma of the liver

Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignant potential.1-3 Hepatic epithelioid hemangioendothelioma (HEHE), a rare sarcoma of the liver, usually appears as multiple nodules involving both hepatic lobes, and can be misdiagnosed as metastatic carcinoma on the basis of its radiologic manifestations. HEHE is composed of neoplastic endothelial cells that closely resemble epithelial cells, and shows a characteristic zoning phenomenon. At the periphery, the tumor cells infiltrate preexisting sinusoids and terminal hepatic venules. The center reveals a marked desmoplastic stromal reaction with dense sclerosis, and these findings may mimic those of cholangiocarcinoma. Therefore, confirming that the tumor is of vascular origin using immunohistochemical staining for endothelial cell markers is critical for accurately diagnosing HEHE. In this issue, we present a case of HEHE in a 44-year-old woman and discuss the histopathologic findings.