Vol. 4, Issue 1, Part B (2024)

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor often presenting as a vulval nodule with intact skin but subcutaneous involvement. Initial resections typically have limited tumor-free margins due to misdiagnosis as benign. Once confirmed histologically, wide excision with 2–3 cm margins is the standard treatment to minimize local recurrence, which is common when margins are inadequate. DFSP rarely metastasizes early, and regional lymphatic involvement is uncommon, making routine lymphadenectomy unnecessary. Histological variants, such as fibrosarcomatous changes, may increase aggressiveness. Microscopically, DFSP infiltrates the dermis with projections extending beyond visible margins. Imatinib, a tyrosine kinase inhibitor, is effective in metastatic cases but does not improve overall survival. Despite the rarity of vulval DFSP, treatment principles align with those for other skin sites, though vulvar excisions may disrupt anatomy. Literature suggests low psychosexual impact post-surgery. In our 25-year-old patient, initial excision failed to achieve clear margins, prompting vulvectomy and bilateral lymphadenectomy. Despite this, her sexual activity was unaffected, and she delivered a child via cesarean section two years later. Four years post-treatment, she remains disease-free, reflecting the importance of early diagnosis, adequate margins, and tailored surgical approaches in DFSP management.