Epithelioid leiomyosarcoma of the vulva: report of a rare case and literature review

Abstract

Leiomyosarcoma of the vulva is a rare soft tissue sarcoma that accounts for approximately 1% of all primary vulvar neoplasms, but it is the most common type of vulvar sarcoma. It usually originates from the smooth muscle within erectile tissue or blood vessel walls, the round ligament, the dartos muscle or the arrector pili muscle. No treatment algorithms have been established to date. Surgical resection is preferred for vulvar leiomyosarcoma. Currently, the recommended surgical method is extensive local resection with a safe surgical margin of at least 2 cm. The use of chemoradiotherapy for vulvar sarcoma remains controversial. This case report describes a 39-year-old female that underwent resection of a vulvar mass in January 2019. Postoperative pathological examination indicated that it was an epithelioid leiomyosarcoma. She presented with tumour recurrence after 43 days. Based on the diagnosis, radical right vulvectomy with a tumour margin of 2 cm was performed. The tumour margin was negative. The patient refused to undergo auxiliary radiotherapy and chemotherapy. The follow-up findings do not indicate any signs of recurrence. In order to avoid recurrence, vulvar epithelioid leiomyosarcomas should be completely resected with a margin of 2 cm at the time of first occurrence.