A rare presentation of Retiform Hemangioendothelioma in sacral area

Abstract

Retiform Hemangioendothelioma is an extremely rare tumor. It was first explained by Calonje et al in 1994. It is a vascular neoplasm of borderline malignancy, mostly seen in young age, and extremities. A 65 years old female patient presented with a recurrent growth in the sacral region, along with skin ulceration. FNAC revealed a cystic lesion with inflammation. Previous biopsy showed granulation tissue only. The growth was surgically removed for the third time and sent to Histopathology lab. Gross examination revealed a partial skin covered tissue mass of 15x10x3 cm. Cut section showed solid cystic growth of 2x2x1.5 cm. Microscopically, proliferating neoplastic elements, vascular in nature, in a net like pattern seen. Monomorphic cells with scant cytoplasm with hobnail nuclei lined the vessels. Scant mitotic figures, lymphocytic infiltration, and focal fibrocollagenous material were seen. Dermis was focally involved. On immunohistochemistry, Ki-67 (low proliferation) and CD 34 were positive. Hence the diagnosis of Retiform Hemangioendothelioma was made.