Background: Dermatofibrosarcoma Protuberans (DFSP) of the vulva is rare. Primary management is surgery. Positive margins are associated with higher recurrence rates. The disease characteristically requires large resections to achieve negative margins, which given the location of vulva is a challenging to devise the least morbid surgery. The management typically requires a multidisciplinary approach for resection or reconstruction.
Case Report: A 25-year-old patient with an incisional biopsy in the left vulvar region diagnosed as DFSP underwent Mohs Micrographic Surgery (MMS) and radical partial vulvectomy with the preservation of all genitals. A 1cm margin resection followed by Complete Circumferential and Peripheral Deep Margin Assessment (CCPDMA) was performed. During MMS, the margins were positive with each resection as it approached the vaginal wall and definitive MMS was aborted. She was then referred back to gynecologic oncology for radical surgical treatment. Subsequent anatomy-sparing radical partial vulvectomy was done and successfully cleared all the positive margins near the vagina and clitoris.
Conclusion: Young patient with DFSP of the vulva is challenging to decide surgical treatment between dermatology and gynecologic oncology. Recommend MMS approach if feasible and in the event of the need of more radical surgery by gynecologic oncology, consider a step-wise approach to preserve as much normal anatomy as possible.
Vivian Hui, Yen-Jen Wang, and Brian Orr*