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Low-Grade Fibromyxoid Sarcoma of the Vulva: A Case Report

VanSandt, Amanda M. DO1; Bronson, Joline MD2; Leclair, Catherine MD2; Mansoor, Atiya MD1; Goetsch, Martha MD2

Journal of Lower Genital Tract Disease: January 2013 - Volume 17 - Issue 1 - p 79–81
doi: 10.1097/LGT.0b013e318256da58
Original Articles

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: vansanda@ohsu.edu

The authors have nothing to disclose, and no funding was received from any source for this study.

©2013The American Society for Colposcopy and Cervical Pathology