Primary epithelioid angiosarcoma of the vulva: A rare tumor at an unusual site

Epithelioid angiosarcoma of the vulva is an exceptionally rare and highly aggressive malignancy originating from endothelial cells. Its etiology remains unclear, though risk factors such as prior radiotherapy and chronic lymphedema have been identified. We report a case of a 44-year-old woman with no significant medical history who presented with a rapidly enlarging, foul-smelling mass on the right labia majora. The tumor measured 30 cm and exhibited central necrosis, ulceration, and bleeding. Radical tumor resection and right inguinal lymphadenectomy were performed, followed by reconstruction using a keystone design perforator island flap and a V-Y advancement flap. Histopathology confirmed a high-grade epithelioid angiosarcoma, positive for ERG, CD31, CD34, podoplanin, EMA, and a Ki-67 proliferation index of 60%. Further treatment consisted of adjuvant radiotherapy (61 Gy) and doxorubicin-based chemotherapy. At one-year follow-up, the patient remained free of recurrence or metastasis. Primary epithelioid angiosarcoma of the vulva is extremely rare, particularly in patients without known risk factors. Management involves radical surgical resection with adjuvant radiotherapy and chemotherapy as indicated. Reporting such cases is essential to enhance understanding and guide evidence-based management of these challenging neoplasms. Keywords: rare tumors, angiosarcoma, keystone design perforator island flap, epitheloid angiosarcoma, gynecologic malignancy, vulvar epitheloid angiosarcoma