A Review for Dermatofibrosarcoma Protuberans

Abstract

Dermatofibrosarcoma protuberans (DFSP) stands as the most prevalent cutaneous sarcoma, marked by slow growth and a protuberant papulonodular appearance. Although rare, its impact is significant, necessitating a comprehensive understanding of its characteristics and optimal management. DFSP typically affects adults, though age variability exists, presenting diagnostic challenges. Histological evaluation, often utilizing CD34 immunohistochemical staining, is crucial for accurate diagnosis. The primary treatment modality remains complete surgical excision, emphasizing the importance of generous margins. Imatinib emerges as a valuable therapeutic option for unresectable or metastatic cases. Complementary approaches such as adjuvant radiation therapy and Mohs surgery enhance the treatment armamentarium, allowing for a tailored, multidisciplinary strategy. Regular follow-up is essential for monitoring and addressing potential complications. While local recurrence is a concern, DFSP generally carries a favorable prognosis, especially with early detection and appropriate intervention. Ongoing research continues to refine treatment paradigms, promising improved outcomes for individuals affected by this distinctive cutaneous sarcoma.