Metastatic dermatofibrosarcoma protuberans of the vulva.

Dermatofibrosarcoma protuberans (DFSP) of the vulva is a rare, low- to intermediate-grade sarcoma of dermal origin. This case report represents the 13th reported patient with vulvar DFSP and the first reported patient with metastatic vulvar DFSP. Although metastasis is rare, the possibility remains that the frequent recurrence of DFSP will eventually metastasize. Therefore, patients must be followed up on a long-term basis to monitor the progress of this potentially lethal cancer.