Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

Dermatofibrosarcoma protuberans of the vulva

Abstract

Objective: Dermatofibrosarcoma protuberans (DFSP) of the vulva is an uncommon soft tissue tumor with fewer than 60 cases reported previously. Distant metastasis is rare with a high propensity for local invasion. Surgical management is the gold standard with adequate margin excision to prevent future recurrences. We report a case of vulvar DFSP requiring three resections to achieve primary clearance. To the best of our knowledge, this is the first case report from India.
Case report: A 35-years-old female, presented with nodular vulvar mass for the third time arising from the upper part of right labia majora for the last year. She had a history of two similar episodes in the past for which excision was done and histopathology confirmed DFSP. The third wide local resection was performed with 3 cm margins and the margins were sent for intra-operative frozen section analysis, which was confirmed clear by the pathologist. The patient has been free of recurrence for two years.
Conclusion: DFSP is a challenging condition presenting as a non-tender nodular mass characterized by local invasion and recurrence. Early diagnosis and appropriate management using wide local excision with accurate margin assessment can achieve optimal results and prevent future recurrences.