The epithelioid hemangioendothelioma is an uncommon vascular neoplasm. Numerous sites of involvement are possible, but these tumors most commonly arise in the soft tissues, liver, and lung. We report a case of epithelioid hemangioendothelioma presenting as a single lesion on the nasal portion of the maxilla. Histologically, the tumor within bone and skin exhibited cords and nests of plump, epithelioid-appearing cells exhibiting rudimentary vascular differentiation within a myxohyaline stroma. Immunohistochemical reactivities for factor VIII-related antigen, CD31, CD34, and vimentin were demonstrated. Wide local excision with close clinical follow-up seems to be the treatment of choice for these tumors.
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