Dermatofibrosarcoma protuberans
Therapeutic area
Dermatology
Rare disease
Yes
- 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
- dermatofibrosarcoma protuberans
- dermatofibrosarcoma
- Dermatofibrosarcoma Protuberans (DFSP)
- Dermatofibrosarcoma Protuberans, Fibrosarcomatous
- Dermatofibrosarcomas of DARIER FERRAND(DFSP)
- DFSP
- familial dermatofibrosarcoma protuberans (subtype)
- metastatic dermatofibrosarcoma protuberans (subtype)
- Severity
- Severe
- Dermatology
- Oncology
- Rheumatology
Sources for recorded values
- MONDO ID
- MONDO:0011934
- Parent condition
- MONDO:0000653
Related records
Broader conditions (20)
- Cancer
- Cancer or benign tumor
- Connective and soft tissue neoplasm
- Connective tissue disorder
- 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
- Neoplastic disease or syndrome
- Sarcoma
- Soft tissue neoplasm
- Soft tissue sarcoma
Narrower conditions (1)
- Pigmented dermatofibrosarcoma protuberans
Trials (41)
- A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors
- 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
Drugs in development (3)
- Doxorubicin hydrochloride Phase 3
- Ifosfamide Phase 3
- Pazopanib Phase 2