Objective: The study aimed to describe a case of low-grade fibromyxoid sarcoma arising from the vulva and to discuss the diagnostic challenges, clinical management, and epidemiology of this rare malignancy.
Case: A 36-year-old woman presented to 3 separate emergency departments with complaints of a painful and slowly enlarging vulvar mass. Eventual gynecologic referral resulted in excision of a 6-cm, noncystic vulvar mass. Pathological diagnosis revealed low-grade fibromyxoid sarcoma. Later, a right radical hemivulvectomy ensured adequate margins, and 2 years later, the patient is free of recurrent and metastatic disease.
Conclusions: Low-grade fibromyxoid sarcoma is a rare malignancy that may present in the lower genital tract. Definitive diagnosis is essential because low-grade fibromyxoid sarcoma may metastasize many years after diagnosis, thereby requiring indefinite clinical surveillance.