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

Lajya Devi Goyal; Priyanka Garg; Manmeet Kaur; Diksha Sharma

doi:10.18502/jfrh.v15i2.6456

Lajya Devi Goyal Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Bathinda, India
Priyanka Garg Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Bathinda, India
Manmeet Kaur Department of Pathology, All India Institute of Medical Sciences, Bathinda, India
Diksha Sharma Guru Gobind Singh Medical College, Faridkot, Punjab, India

DOI: https://doi.org/10.18502/jfrh.v15i2.6456

Keywords: Dermatofibrosarcoma; Vulva; Margins of Excision; Local Neoplasm Recurrence

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.