A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report

Abstract

Background Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin with high local recurrence rate that rarely presents in the vulva. It affects adults between the second and fifth decade of life and the involved areas are most frequently the trunk, proximal extremities, head and neck. Case Presentation A 45-years-old Para 4 women presented with two raised masses on the right part of vulva for the last 17-month duration which increase in size for the last 6-month. First, incisional biopsy was taken from the mass and diagnosed as “Suggestive of leiomyoma”. Following to this, excision of the whole mass was done and sent for histopathologic examination. The final histopathologic diagnosis became DFSP with the classic microscopic picture of a storiform and honeycomb pattern of monomorphic bland spindle cells. Conclusion Dermatofibrosarcoma protuberans infrequently involves the vulva and should be considered in the differential diagnosis of other spindle cell lesions presenting in this unusual site.