What is it about?

Dermatofibrosarcoma protuberans (DFSP) is a rare skin-and-soft-tissue cancer that grows locally and only rarely spreads. This review explains, in plain terms, how DFSP is diagnosed and treated. It describes the tell-tale laboratory features used to confirm DFSP, why specialised surgery that checks all edges under the microscope (Mohs surgery or similar) gives the lowest risk of the tumour coming back, and when standard wide local excision is appropriate. It also outlines when radiotherapy can help, for example after close or positive margins, and how targeted tablets such as imatinib can shrink tumours that are hard to remove or have spread. A higher-risk form called fibrosarcomatous DFSP is explained, with practical advice on follow-up and reconstruction after surgery.

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Why is it important?

Patients with DFSP often face repeated operations because the tumour sends tiny finger-like strands beyond what is visible. This paper brings together the best available evidence to show which treatments cut that risk most, balancing cancer control with good cosmetic and functional outcomes. It clarifies when to choose Mohs surgery, when radiotherapy adds value, and when tablets like imatinib are likely to work. For patients and clinicians, it offers a clear roadmap that can reduce unnecessary procedures, speed curative treatment, and guide care for the uncommon aggressive variant.

Perspectives

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma with an incidence of 0.008 to 0.045 cases per 100,000 per year, accounting for less than 1% of all soft tissue sarcomas. It mainly affects adults aged 30–50 years, usually on the trunk and proximal extremities. DFSP is Locally aggressive, with metastases occurring in up to 5% of cases, typically < 1%. Diagnosis is based on histology, including CD34 antigen expression and COL1A1-PDGFB fusion detection by FISH or RT-PCR. Mohs micrographic surgery is the mainstay of treatment, ensuring clear margins to minimise recurrence. Radiotherapy is used as adjuvant or preoperative therapy to improve Local control. Imatinib, a tyrosine kinase inhibitor, is highly effective in unresectable or metastatic DFSP, especially in cases with PDGFB mutations, achieving disease control in over 70% of patients and partial responses in 36%. The 10-year overall survival rate is 90.7%, although the fibrosarcomatous variant (DFSP-FS) has worse outcomes, with a 5-year Local progression-free survival rate of 33%. Rare metastases to the lungs, lymph nodes or brain are treated surgically; chemotherapy remains ineffective. We comprehensively reviewed the clinical data regarding DFSP, highlighting subtype differences (myxoid, pigmented, myoid, granular cell, sclerosing, atrophic, and fibrosarcomatous), as well as reconstruction methods.

Piotr Remiszewski
Maria Sklodowska- Curie National Research Institute of Oncology

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This page is a summary of: Dermatofibrosarcoma Protuberans (DFSP): Current Treatments and Clinical Trials, Current Treatment Options in Oncology, October 2025, Springer Science + Business Media,
DOI: 10.1007/s11864-025-01348-y.
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