Background: Dermatofibrosarcoma protuberans (DFSP), a low-grade sarcoma of dermal origin, rarely is found on the vulva. Only 16 cases of DFSP of the vulva have been described.
Cases: Two patients with long histories of primary vulvar dermatofibrosarcoma protuberans are presented. Both required multiple excisions to resect the tumor completely. The patients remain without evidence of disease after long-term follow-up. One patient is among the youngest recorded. All published cases of vulvar DFSP are reviewed.
Conclusions: Both our experience and that reported in previous cases indicate that wide local excision is required in the treatment of vulvar DFSP.