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Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22).
Biomarker and diagnostic research for dermatofibrosarcoma protuberans has been reported in the published literature.
1 FDA-approved treatment is available for dermatofibrosarcoma protuberans, including IMATINIB MESYLATE (GLEEVEC, approved 2003).
Brand Name | Generic Name | Mechanism | Approved | Market Status |
|---|---|---|---|---|
Estimated prevalence: 1-5 in 10,000 (Uncommon).
7 clinical trials registered, 2 recruiting. Interventions under study include drug therapy, procedural interventions, and other interventions. Pipeline includes 3 PHASE2, 3 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT02180867](https://clinicaltrials.gov/study/NCT02180867) |
Data assembled from 6 of 12 sources · Last updated Sep 18, 2026, 4:02 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
GLEEVEC
IMATINIB MESYLATE |
— |
2003 |
Available |
7 trials found
Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery |
PHASE2 |
National Cancer Institute (NCI) |
UNKNOWN |
[NCT06610071](https://clinicaltrials.gov/study/NCT06610071) | NIFR Image-guided Surgery for Malignant Soft Tissue Tumor With Low-dose SWIG Technique | PHASE1 | The Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College | UNKNOWN |
[NCT06526897](https://clinicaltrials.gov/study/NCT06526897) | Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma | NA | ECOG-ACRIN Cancer Research Group | NOT_YET_RECRUITING |
[NCT04332874](https://clinicaltrials.gov/study/NCT04332874) | A Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg | PHASE2 | Memorial Sloan Kettering Cancer Center | RECRUITING |
[NCT06789172](https://clinicaltrials.gov/study/NCT06789172) | A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors | PHASE1 | Epkin | UNKNOWN |
210 publications have been identified in PubMed for dermatofibrosarcoma protuberans. Kisho has analyzed 121 by research type. Research spans Case Report / Case Series (35%), Review / Meta-Analysis (30%), and Basic Science / Preclinical (10%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 42 | 35% |
Research summaries | 36 | 30% |
Laboratory research | 12 | 10% |
Disease patterns and progression | 10 | 8% |
Other research | 9 | 7% |
Testing and diagnosis research | 9 | 7% |
Clinical study results | 3 | 2% |
Janczewski LM (2026). [PMID: 41224441](https://pubmed.ncbi.nlm.nih.gov/41224441/). *Hematol Oncol Clin North Am*. [Review / Meta-Analysis]
Özdemir İ (2026). [PMID: 41555603](https://pubmed.ncbi.nlm.nih.gov/41555603/). *Australas J Dermatol*. [Review / Meta-Analysis]
Cloutier JM (2026). [PMID: 41755369](https://pubmed.ncbi.nlm.nih.gov/41755369/). *Histopathology*. [Basic Science / Preclinical]
Zhuang N (2026). [PMID: 41698654](https://pubmed.ncbi.nlm.nih.gov/41698654/). *J Int Med Res*. [Epidemiology / Natural History]
Kato M (2026). [PMID: 41888635](https://pubmed.ncbi.nlm.nih.gov/41888635/). *J Dermatol*. [Review / Meta-Analysis]
Zhang H (2026). [PMID: 41790694](https://pubmed.ncbi.nlm.nih.gov/41790694/). *Medicine (Baltimore)*. [Case Report / Case Series]
Cloutier JM (2026). [PMID: 41711154](https://pubmed.ncbi.nlm.nih.gov/41711154/). *Genes Chromosomes Cancer*. [Basic Science / Preclinical]
Neyaz A (2026). [PMID: 41025914](https://pubmed.ncbi.nlm.nih.gov/41025914/). *Histopathology*. [Diagnostic / Biomarker]
Yang X (2026). [PMID: 41907634](https://pubmed.ncbi.nlm.nih.gov/41907634/). *Front Oncol*. [Case Report / Case Series]
Meyer A (2026). [PMID: 42141704](https://pubmed.ncbi.nlm.nih.gov/42141704/). *Int J Surg Pathol*. [Review / Meta-Analysis]
AI-curated news mentioning dermatofibrosarcoma protuberans
Updated Apr 6, 2026
A case report details a rare instance of primary synovial sarcoma of the breast that initially presented as dermatofibrosarcoma protuberans. This finding contributes to the understanding of differential diagnoses in rare breast tumors.