Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology

Authors:
Jeremy Bordeaux Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute

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Rachel Blitzblau Duke Cancer Institute

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Sumaira Z. Aasi Stanford Cancer Institute

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Murad Alam Robert H. Lurie Comprehensive Cancer Center of Northwestern University

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Arya Amini City of Hope National Medical Center

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Kristin Bibee Johns Hopkins Kimmel Cancer Center

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Diana Bolotin The UChicago Medicine Comprehensive Cancer Center

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Pei-Ling Chen Moffitt Cancer Center

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Carlo M. Contreras The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute

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Dominick DiMaio Fred & Pamela Buffett Cancer Center

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Jessica M. Donigan Huntsman Cancer Institute at the University of Utah

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Jeffrey M. Farma Fox Chase Cancer Center

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Karthik Ghosh Mayo Clinic Comprehensive Cancer Center

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Kelly Harms University of Michigan Rogel Cancer Center

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Tejesh Patel The University of Tennessee Health Science Center

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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.

Discussion Writing Committee Members:

Jeremy Bordeaux, Rachel Blitzblau, Beth McCullough, and Sara Espinosa

To view disclosures of external relationships for the NCCN Guidelines panel, go to https://www.nccn.org/guidelines/guidelines-panels-and-disclosure/disclosure-panels

The complete and most recent version of these guidelines is available free of charge at NCCN.org.

NCCN CATEGORIES OF EVIDENCE AND CONSENSUS

Category 1: Based upon high-level evidence, there is uniform NCCN consensus that the intervention is appropriate.

Category 2A: Based upon lower-level evidence, there is uniform NCCN consensus that the intervention is appropriate.

Category 2B: Based upon lower-level evidence, there is NCCN consensus that the intervention is appropriate.

Category 3: Based upon any level of evidence, there is major NCCN disagreement that the intervention is appropriate.

All recommendations are category 2A unless otherwise indicated.

NCCN CATEGORIES OF PREFERENCE

Preferred intervention: Interventions that are based on superior efficacy, safety, and evidence; and, when appropriate, affordability.

Other recommended intervention: Other interventions that may be somewhat less efficacious, more toxic, or based on less mature data; or significantly less affordable for similar outcomes.

Useful in certain circumstances: Other interventions that may be used for selected patient populations (defined with recommendation).

All recommendations are considered appropriate.

NCCN recognizes the importance of clinical trials and encourages participation when applicable and available.

Trials should be designed to maximize inclusiveness and broad representative enrollment.

PLEASE NOTE

The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines® is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use, or application and disclaims any responsibility for their application or use in any way.

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