Dermatofibrosarcoma Protuberans: A Diagnostic and Management Conundrum-A Retrospective Study from a Tertiary Care Center in South India.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with low metastatic potential with high local recurrence rates, arising in the dermis and often mimics other benign skin conditions. FS-DFSP is a more aggressive variant, associated with higher metastatic potential. While wide local excision is considered the primary modality of treatment, patients with high risk features would necessitate adjuvant therapy. Our study looked at challenges in diagnosing and managing DFSP. This retrospective analysis involved 50 patients who had surgical management of DFSP at a tertiary care center in South India from April 2012 to September 2024. In a study of 50 patients, most were male, with a median age of 40 years. Tumors had a median size of 4 cm, primarily located in the anterior abdominal wall. Eight four percent underwent wide local excision with a 3 cm margin; 33 patients had primary closure, while eight required flap coverage. Post-operative complications occurred in 11 patients, mainly surgical site infections. FS-DFSP was observed in 14 cases, and 24 patients received adjuvant radiation therapy due to close margins or recurrence. Only two patients were treated with adjuvant imatinib. We had only one recurrence in our study. Pre-operative biopsy and imaging can help prevent inadequate excisions, reducing recurrences and the need for re-excision and adjuvant therapy. Accepted surgical margins range from 2 to 3 cm, but wider margins necessitate flap covers and wound complications. Adjuvant therapy in select patients with local recurrence, close surgical margins, and aggressive histological types can improve outcomes.