Vulvar Dermatofibrosarcoma protuberance in Iran and a narrative review of literature: A first case report

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor that originates from deep layers of the dermis and mainly is common in young adults to middle age. This tumor is rare in the vulva. CASE PRESENTATION: A 53-year-old multiparous menopause female had complained of asymptomatic swelling of the right labia major. Dermatofibrosarcoma protuberans was reported in primary tumor resection. Six months later, rapid growing mass recurred in the vulva. The patient underwent radical Vulvectomy and resection of the margin of about 2-3 cm along with bilateral Inguinofemoral lymphadenectomy. The margins of the mass were negative in the frozen section. Microscopic examination revealed that hypercellular neoplasm in dermis comprising monomorphic spindle cells with high mitotic activity, some hyperchromatic nuclei arranged in palisading fashion. Microscopic and IHC study confirmed the conversion of dermatofibrosarcoma protuberans to fibrosarcoma. CONCLUSION: This case was presented due to the rarity of dermatofibrosarcoma protuberance in the vulva and pathologic conversion to fibrosarcoma.