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Keywords

Epithelioid sarcoma, Proximal type epithelioid sarcoma, SMARCB1/INI1, Rhabdoid cells

Abstract

A vulva biopsy from a 22-year-old woman revealed the rare and aggressive epithelioid sarcoma, characterized by distinct histomorphology and a specific immunohistochemical profile. This case report highlights its clinical features, etiology, and diagnostic histopathology, emphasizing the crucial role of immunostaining for an accurate diagnosis and effective management.

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