Abstract
KEY CLINICAL MESSAGE: Dermato fibrosarcoma protuberans presents challenges in diagnosis and treatment, especially in children. Awareness of its aggressive local recurrence and its potential for multifocal presentation is crucial. Wide surgical resection with adequate margins remains the basis of management, in association with regular follow-up of affected patients. ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma, particularly uncommon in children. We report the case of a 13-year-old boy who initially presented with a firm mass on the left foot, later diagnosed as DFSP following histological and immunohistochemical analysis. The tumor was surgically excised with a wide margin, and a skin graft was used for coverage. Despite an initial favorable outcome, a new DFSP lesion developed at the proximal left thigh 1 year later, requiring further wide surgical excision and coverage with a tensor fascia lata flap. Both sites remained free of recurrence at one-year follow-up. This case underscores the importance of long-term vigilance in managing DFSP due to the potential risk of recurrence and multifocal involvement.