Early-onset epithelioid angiosarcoma: Diagnostic enigma, a rare case report

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Epithelioid angiosarcoma (AS) is a rare vascular tumor affecting more commonly men in their seventh decade. We are reporting a case of epithelioid AS affecting a male patient in his third decade. He presented with a well-defined ulcerated plaque with an elevated border on the scalp. Histopathology showed sheets of epithelioid cells infiltrating the dermis, formation of slit-like vascular spaces, and cellular atypia. Immunohistochemistry showed positive CD31, D2-40, and FLI-1 and negative cytokeratin and epithelial membrane antigen. With these findings, a diagnosis of epithelioid AS was made. This case highlights the rare occurrence of this condition in younger age group.