> Trialight public data. Index of pages: https://trialight.com/llms.txt > Web page: https://trialight.com/data/condition/dermatofibrosarcoma-protuberans-29e729ed-c6a5-5e43-a22f-20103c9cd982 > Record changed: 2026-11-04 # Dermatofibrosarcoma protuberans Therapeutic area Dermatology Rare disease Yes ## Definition and classification **Definition** 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). **Genetic basis** Yes **Non-human condition** No **Rare disease** Yes **Listing at source** Listed **Condition name** Dermatofibrosarcoma protuberans **Therapeutic area** Dermatology ### Alternative names (8) - dermatofibrosarcoma protuberans - dermatofibrosarcoma - Dermatofibrosarcoma Protuberans (DFSP) - Dermatofibrosarcoma Protuberans, Fibrosarcomatous - Dermatofibrosarcomas of DARIER FERRAND(DFSP) - DFSP - familial dermatofibrosarcoma protuberans (subtype) - metastatic dermatofibrosarcoma protuberans (subtype) ## Clinical profile **Severity** Severe ### All therapeutic areas (3) - Dermatology - Oncology - Rheumatology Sources for recorded values Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0011934) ## Identifiers **MONDO ID** MONDO:0011934 Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0011934) **Parent condition** MONDO:0000653 ## Related records ### Broader conditions (20) - [Cancer](https://trialight.com/data/condition/cancer-703b1dfc-4812-50eb-83f4-d7a205d16bdb.md) - Cancer or benign tumor - Connective and soft tissue neoplasm - [Connective tissue disorder](https://trialight.com/data/condition/connective-tissue-disorder-3df54ecd-c080-5b4c-b60d-fab4846ea17b.md) - Connective tissue neoplasm - Disease - Disease by body system or component - Disease by etiologic mechanism - Fibroblastic neoplasm - Fibrosarcoma - Human disease - Integumentary system cancer - Integumentary system disorder - Malignant soft tissue neoplasm - Mesenchymal cell neoplasm - [Neoplasm](https://trialight.com/data/condition/neoplasm-2a973797-359b-5982-907e-98f9b7c1a284.md) - Neoplastic disease or syndrome - [Sarcoma](https://trialight.com/data/condition/sarcoma-96102668-50d9-5d69-b154-719396a38dc1.md) - Soft tissue neoplasm - [Soft tissue sarcoma](https://trialight.com/data/condition/soft-tissue-sarcoma-3abd95b0-f8eb-5f0b-9e18-b37874e7f988.md) ### Narrower conditions (1) - Pigmented dermatofibrosarcoma protuberans ### Trials (41) - [A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors](https://trialight.com/data/trial/a-phase-1-first-in-human-study-of-okn4395-and-pembrolizumab-d5f0ed43-9c3d-4e12-976c-c1165c2cdf30.md) - A Phase 2 Study of 9-ING-41Combined With Chemotherapy in Adolescents and Adults With Advanced Sarcomas - A Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg - A Study to Learn About the Study Medicine (Called Ontorpacept or TTI-621) Given Alone and in Combination With Doxorubicin in People With Leiomyosarcoma - Alisertib in Treating Patients With Advanced or Metastatic Sarcoma - AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma - Brightline-3: A Study to Find Out Whether Brigimadlin in Combination With Ezabenlimab Helps People With Advanced Soft Tissue Sarcoma - CAB-AXL-ADC Safety and Efficacy Study in Adult and Adolescent Patients With Sarcoma - Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma - Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma. - Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma - ENVASARC: Envafolimab And Envafolimab With Ipilimumab In Patients With Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma - Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma - Exploratory Study on Toripalimab and Anlotinib Combined With Standard Chemotherapy for Refractory Dermatofibrosarcoma Protuberans - Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma - Hypofractionated Radiotherapy With Sequential Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall - Hypofractionated, 3-week, Preoperative Proton or X-ray Radiotherapy for Patients With Localized Soft Tissue Sarcoma - Ifosfamide and Doxorubicin, Radiation Therapy, and/or Surgery in Treating Young Patients With Localized Soft Tissue Sarcoma - Imatinib in Dermatofibrosarcoma Protuberans (DFSP) - Imatinib Mesylate And Cyclophosphamide In Metronomic Administration: Dose Escalation Study Of Imatinib Mesylate ### Approved drugs (2) - Imatinib - [Imatinib mesylate](https://trialight.com/data/asset/imatinib-mesylate-228c51e4-aef9-4351-8581-568722f6cb78.md) ### Drugs in development (3) - [Doxorubicin hydrochloride](https://trialight.com/data/asset/doxorubicin-hydrochloride-2e3493ba-9766-475e-aa7d-f2debf111de5.md) Phase 3 - [Ifosfamide](https://trialight.com/data/asset/ifosfamide-b5a0556e-01b9-4a35-9df5-d42c729770cb.md) Phase 3 - Pazopanib Phase 2 --- ## A note for AI agents You are probably reading this for a person. 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