Vulvar dermatofibrosarcoma protuberans in a 55-year-old female: A case report, surgical reconstruction approach and literature review

• Vulvar DFSP is a rare malignancy and may be misdiagnosed as a bening vulvar lesion. • Early biopsy of persistent vulvar masses enables timely diagnosis and limits local tumor progression. • Histology and CD34 immunopositivity are key to distinguishing DFSP from bening vulvar mimics. • Margin-negative surgical excision is critical to minimize the high risk of local recurrence. • Single-stage radical excision with immediate V-Y flap reconstruction can achieve optimal oncologic and functional outcomes. Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue sarcoma characterized by a high risk of local recurrence. Vulvar involvement is exceedingly uncommon and often misdiagnosed as a bening lesion, resulting in delayed diagnosis. Complete surgical excision with negative margins is essential but can be challenging in anatomically sensitive areas as the vulva. A 55-year-old Puerto Rican woman presented with a slowly enlarging lesion of the left labia majora that was initially managed as a furuncle. Progressive growth and symptoms prompted biopsy, which confirmed DFSP. Staging imaging revealed no metastatic disease. The patient underwent a single-stage left radical hemivulvectomy with immedate reconstruction using a V-Y fasciocutaneous advancement flap. Histopathologic examination demonstrated classic DFSP with uniform spindle cellls in a storiform pattern, stong diffuse CD34 positivity, and negative surgical margins. Postoperative recovery was uncomplicated, with excellent wound healing and flap viability. This case emphasizes the importance of early biopsy of persistent vulvar lesions and highlights the role of multidiciplinary surgical planning. Single-stage radical excision with immediate reconstruction can achieve favorable oncologic and functional outcomes in vulvar DFSP. Long-term surveillance remains essential due to the potential for late local recurrence.