DOI: https://doi.org/10.36347/sjmcr.2025.v13i09.025

Pages: 2054-2058

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchymal skin tumor characterized by slow growth, local aggressiveness, and a low metastatic potential. Facial involvement is uncommon, posing diagnostic and therapeutic challenges. Case Presentation: We report the case of a 38-year-old male presenting with a progressively enlarging mass on the right cheek. Imaging and biopsy confirmed DFSP, and molecular testing identified the COL1A1-PDGFB fusion. Given the lesion’s size, muscular infiltration, and potential functional and aesthetic morbidity, neoadjuvant imatinib (400 mg/day) was administered for three cycles, achieving a 40% volumetric reduction. This allowed a wide local excision with clear margins (R0) and local flap reconstruction, preserving facial function and aesthetics. At 12 months follow-up, the patient remained in complete remission without local recurrence or metastasis. Conclusion: This case illustrates the efficacy of neoadjuvant imatinib in locally advanced DFSP of the head and neck, facilitating conservative surgical management. Molecular confirmation of COL1A1-PDGFB fusion is essential to guide targeted therapy. Lifelong surveillance remains crucial due to the risk of late local recurrence.