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.
Vulvar masses not conforming to the usual clinical paradigm require a broadened clinical differential diagnosis, including low-grade fibromyxoid sarcoma and other rare lesions.
1Departments of Pathology and 2Obstetrics and Gynecology, Oregon Health and Science University, Portland, OR
Reprint requests to: Amanda M. VanSandt, DO, Department of Pathology, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Mail Code L-113, Portland, OR 97239. E-mail: firstname.lastname@example.org
The authors have nothing to disclose, and no funding was received from any source for this study.