Abstract

Hemangioendothelioma was termed by Borrmann who described the concept of vascular neoplasms with intermediate or low malignant potential. The epithelioid hemangioendothelioma (EH) type was first proposed by Sharon Weiss and Franz Enzinger in 1982 as an angiocentric vascular neoplasm characterized by proliferation of epithelioid endothelial cells in cords, strands or small nests with abundant eosinophilic cytoplasm embedded in a myxohyaline stroma. Hemangioendothelioma is classified into three types, kaposiform, hobnail and epithelioid. EH can occur at almost any age, especially middle-aged patients and is primarily diagnosed in soft tissues of the extremities, lung, liver, bones and skin. EH is rare in the vulva with two cases being reported so far. Herein, we report a case of vulvar EH in a 38 year old female which presented with a 3 cm vulvar mass showing a proliferation with myxohyaline stroma in the center surrounded by nests of epithelioid to stellate to spindle cells.

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