Dermatofibrosarcoma protuberans of the vulva: a case report

Area of Science:

• Oncology
• Dermatology
• Surgical Pathology

Background:

• Dermatofibrosarcoma protuberans (DFSP) is a rare, low-to-intermediate grade sarcoma originating in the dermis.
• While local recurrence is common, distant metastasis is infrequent.
• Complete surgical excision is the primary treatment modality for DFSP.

Purpose of the Study:

• To report a challenging case of vulvar dermatofibrosarcoma protuberans (DFSP) requiring multiple surgical resections.
• To highlight the utility of intraoperative frozen section analysis in achieving clear margins for DFSP.
• To emphasize the importance of wide and deep local excision for managing primary and recurrent DFSP.

Main Methods:

• A case report of a 56-year-old woman with a recurrent vulvar DFSP.
• Description of four sequential surgical excisions to achieve tumor clearance.
• Inclusion of intraoperative frozen section analysis to assess surgical margins during the final resection.

Main Results:

• The patient underwent three initial excisions with positive margins for DFSP.
• The fourth excision, guided by intraoperative frozen section analysis, achieved clear margins.
• The patient remained recurrence-free for 15 months postoperatively.

Conclusions:

• Wide and deep local excision is crucial for managing both primary and recurrent DFSP.
• Intraoperative frozen section analysis is a valuable tool for confirming clear surgical margins, potentially reducing the need for multiple resections.
• Achieving complete tumor resection is critical for preventing recurrence in DFSP.