A case of childhood epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a unique tumor, mainly of adults, which is characterized by epithelioid or histiocytoid endothelial cells. It is a low-grade malignant vascular tumor first described as a distinctive entity in 1982 by Weiss and Enzinger. An 8-year-old girl presented with an approximately 3-year history of an erythematous plaque on the left frontal area of the scalp. The diagnosis of EHE confined to the skin was made by clinicopathologic and radiologic evaluation. To our knowledge, there are only 5 reported cases of EHE with skin involvement, 2 of which showed concomitant systemic involvement, and all occurred in adults. Our case is the fourth reported case of EHE confined to the skin and the first to describe childhood onset of isolated cutaneous EHE.