Work

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

Year: 2026

Type: article

Abstract: Background: 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 benign lesion, resulting in delayed diagnosis. Complete surgical excision with negative margins is essential but can be challenging in anatomically sensitive areas as the vulva. Case Presentation: 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. Conclusion: 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.

Source: Gynecologic Oncology Reports

DOI: 10.1016/j.gore.2026.102024

Authors: Amanda Detrés, Itzamar Pastrana, Marjolaine Suárez, Ricardo Gómez

Institution: Hospital Episcopal San Lucas

Language: English

Topic: Soft tissue tumor case studies

Subfield: Rheumatology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $930