Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology
Jeremy S. Bordeaux, Rachel Blitzblau, Sumaira Z. Aasi, Murad Alam, Arya Amini, Kristin Bibee, Diana Bolotin, Pei-Ling Chen, Carlo M. Contreras, Dominick J. DiMaio, Jessica M. Donigan, Jeffrey M. Farma, Karthik Ghosh, Kelly L. Harms, Nicole R. LeBoeuf, John N. Lukens, Susan Manber, Lawrence A. Mark, Theresa Medina, Kishwer S. Nehal, Paul Nghiem, Kelly Olino, György Paragh, Soohyung Park, Tejesh Patel, Jason T. Rich, Ashok R. Shaha, Bhavina Sharma, Yemi Sokumbi, Divya Srivastava, Valencia D. Thomas, Courtney M. Tomblinson, Puja Venkat, Yaohui Xu, Siegrid S. Yu, Mehran Yusuf, Beth McCullough, Sara Espinosa
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. Fibrosarcomatous DFSP is an aggressive variant with an increased risk for local recurrence and metastasis. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP.