Vulvar dermatofibrosarcoma protuberans: a case series

Dermatofibrosarcoma protuberans (DFSP) is a rare, superficial low - to intermediate-grade sarcoma that typically presents as an asymptomatic slow-growing indurated dermal plaque. Although DFSP has a relatively high local recurrence rate, distant metastasis is rare. DFSP primarily occurs on the trunk and extremities but is seldom reported in the vulva. Due to its rarity, the clinical characteristics, pathological diagnosis, prognosis, and optimal management of vulvar DFSP remain poorly characterized. We retrospectively analyzed vulvar DFSP cases from our institution (January 2010 - January 2023). Clinical data and follow-up were obtained from hospital records, and imaging studies were reviewed via the picture archiving and communication system. Seven patients were included, with a median symptom-onset age of 44.3 years (range, 27-73). Patients typically presented with firm, asymptomatic masses. The labia majora was most commonly affected (n = 6, 85.7%). Tumor size averaged 4.3 cm (range, 2.0-6.5). All patients underwent excisional biopsy followed by wide local excision; none received lymphadenectomy. Surgical margins were documented in six patients, with negative margins achieved in five after initial wide excision. Over a mean follow-up of 27.3 months (range, 12-54), one patient (14.3%) experienced local recurrence. No recurrence occurred in patients with negative margins. Vulvar DFSP predominantly affects young and middle-aged women, manifesting as vulvar masses of variable size. Wide local excision is the primary treatment. While vulvar DFSP has a propensity for local recurrence, widely negative margins appear protective. Long-term follow-up is recommended to monitor for recurrence.