Recurrent vulvar tumor consistent with myxoid dermatofibrosarcoma protuberans in a resource-limited setting: A case report from Somalia.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive soft tissue tumor, and its occurrence in the vulva is exceptionally rare. We describe the case of a 35-year-old woman who presented with a recurrent vulvar mass one year after excision of a lesion previously diagnosed as aggressive angiomyxoma. Histopathological examination of the recurrent lesion demonstrated spindle cell proliferation within a myxoid stroma, focal storiform architecture, and honeycomb infiltration of the subcutaneous adipose tissue, findings that were morphologically consistent with myxoid dermatofibrosarcoma protuberans. Because immunohistochemical and molecular testing were unavailable, definitive pathological confirmation could not be established. The patient underwent wide local excision followed by reconstruction with a split-thickness skin graft. Histopathological examination demonstrated involvement of the deep surgical margin. After multidisciplinary discussion, adjuvant imatinib (400 mg once daily) was initiated for 12 months because additional surgery was considered likely to result in significant anatomical and functional morbidity. No evidence of local recurrence was observed during 12 months of follow-up. This case highlights the diagnostic uncertainty and treatment challenges associated with rare vulvar mesenchymal tumors in resource-limited settings and underscores the importance of clinicopathologic correlation, individualized multidisciplinary management, and long-term clinical follow-up.