RESUMO
Epithelioid hemangioendothelioma is a rare vascular tumor of endothelial origin that commonly occurs in the deep soft tissue, bone, lung, and liver, although cutaneous forms have rarely been reported. Tufted angioma (TA) is a rare slowly progressive vascular lesion characterized by multiple capillary tufts with the characteristic "canon-ball" appearance scattered throughout the dermis. A 35-year-old male presented a single, 2.5x1.5 cm sized, purpuric mass on the back. A biopsy specimen showed multiple lobules composed of epithelioid endotheial cells with intracytoplasmic vacuoles in a hyalinized and myxoid stroma, and large dilated vessels. The tumor cells were positive for CD31, CD34, and factor VIII-related Antigen, and the crescent shaped dilated vessels were negative for D2-40. We could not find any metastatic lesions on radiologic examinations. The patient was diagnosed with primary cutaneous epithelioid hemangioendothelioma. Herein, we present a rare and interesting case of primary cutaneous epithelioid hemangioendothelioma resembling TA histopathologically.