Low-grade fibromyxoid sarcoma of the vulva presenting as a cystic mass: A case report and review of literature

Low-grade fibromyxoid sarcoma (LGFMS) is a tumor with a propensity for late recurrence which is rarely described in the vulva. A 22-year-old woman presented with a growing right vulvar cystic mass that had been present for 2 months. She underwent surgical wide excision. The final pathologic diagnosis revealed LGFMS of the vulva and a right radical hemivulvectomy with negative margins was performed. The patient has not experienced a local or metastatic recurrence after 2-years follow- up. Despite being rare, LGFMS of the vulva should be taken into account when making a diagnosis of vulvar lesions. Definite diagnosis is based on pathological examination. MUC4 positivity is characteristically expressed. To prevent future recurrences, radical excision is necessary. •Low-grade fibromyxoid sarcoma is very rare in the vulva.•Low-grade fibromyxoid sarcoma can be misdiagnosed due to its benign appearance clinically.•The golden standard treatment of this tumor is surgery with negative margins.