Case Study of Dermatofibrosarcoma Protuberans and Its Management

Background: Dermatofibrosarcoma Protuberans (DFSP) is a rare, low-to-intermediate grade soft tissue sarcoma of dermal origin characterized by slow growth, local aggressiveness, and a high propensity for local recurrence despite its low metastatic potential. It commonly presents as a painless, indurated cutaneous plaque that gradually progresses into nodular or protuberant masses, most frequently affecting the trunk and proximal extremities. Diagnosis is established through histopathological examination demonstrating spindle cell proliferation in a storiform pattern, supported by immunohistochemical positivity for CD34. Surgical excision remains the cornerstone of treatment, with wide local excision or Mohs micrographic surgery providing optimal local control through complete margin clearance. In cases of unresectable, recurrent, or metastatic disease, targeted therapy with imatinibmesylate has shown significant clinical benefit due to the characteristic COL1A1-PDGFB fusion gene associated with DFSP. Long-term follow-up is essential because of the risk of local recurrence, particularly in cases with inadequate surgical margins. Early diagnosis, appropriate surgical management, and vigilant surveillance are critical in achieving favorable outcomes and minimizing morbidity associated with this uncommon neoplasm.