Epithelioid hemangioendothelioma of the maxilla: case report and review of literature.

Abstract

Epithelioid Hemangioendothelioma (EH) is a rare, low-grade malignant vascular tumor, first defined as a soft tissue tumor by Enzinger and Weiss in 1982. The tumor was later reported in virtually all sites, including liver, lung, brain, and bone. The epithelioid neoplastic cells are of endothelial origin, staining positive for Factor VIII and CD-34. In our review of EH of bone medline literature, and including the present case report, a total of 73 cases were found. Forty-one (56%) patients had multifocal bone involvement and thirty-two (44%) patients had solitary EH of bone. Resection was the primary recommended treatment. Visceral involvement appears to affect prognosis and survival adversely. We report a case of EH of the maxilla, which occurred in a 32-year-old male.