Dermatofibrosarcoma Protuberans (DFSP) of the Vulva: Mohs Micrographic Surgery or Radical Vulvectomy? - Case Report
2020
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.
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