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Disease Information for Dermatofibrosarcoma Protruberans

Clinical Manifestations
Signs & Symptoms: Painful subcutaneous nodules; Skin nodules/Subcutaneous nodules
Disease Progression: Course/Chronic disorder; Course/Chronic only; Course/Mild illness; Prognosis/Full recovery usual
Laboratory Tests
Abnormal Lab Findings (Non Measured): Cells/Tissue CD34 Positive
Diagnostic Test Results
Pathology: BX/Skin biopsy/Abnormal
Associated Diseases & Rule outs
Associated Disease & Complications: Dermatofibroma; Dermatofibrosarcoma Protruberans; Sarcomas/soft-tissue/others; Spindle cell tumor/sarcoma
Disease Mechanism & Classification
Class: CLASS/Fibroblast Fibrocyte
: CLASS/Dermatologic/Subcutaneous (category)
Pathophysiology: Pathophysiology/Chromosomal translocation; Pathophysiology/Gene locus Chromosome 17; Pathophysiology/Gene locus chromosome 22; Pathophysiology/Metastasis slow-growing resectable
: Pathophysiology/COL1A1/PDGFB translocation; Pathophysiology/Collagen gene (COL1A1) Mutation translocation; Pathophysiology/False Collagen Becomes PDGFB; Pathophysiology/Gene locus 17/22 translocation; Pathophysiology/PDGFR (platelet derived Growth factor receptor) expression; Pathophysiology/Somatic mutation Tumor Tissue
Process: PROCESS/INCIDENCE/Rare disease (ex); PROCESS/Malignancy/cancer (ex); PROCESS/Neoplastic (category); PROCESS/Sarcoma (ex); PROCESS/Sarcoma/Fair Prognosis/No Metastasis usually
: PROCESS/Somatic mutation/not hereditary
Synonyms
Synonym: Dermatofibroma protuberans, Dermatofibrosarcom protuberans, Dermatofibrosarcoma, Dermatofibrosarcoma protuberans, Dermatofibrosarcoma protuberans (disorder), DFSP, DFSP Dermatofibrosarc protru, DFSP Dermatofibrosarcoma protruberans, PDGFB COL1A1 FUSION, protuberans dermatofibrosarcoma, Synonym/DFSP
Treatment
Drug Therapy - Indication: RX/Chemotherapy(cancer); RX/Imatinib (Gleevec/Glivec)
Surgical Procedures or Treatments: SX/Biopsy; SX/Surgery
: SX/MOHS Micrographic Surgery
Other Treatments: TX/Radiotherapy.
Definition: Be the first to add a definition for Dermatofibrosarcoma Protruberans
External Links Related to Dermatofibrosarcoma Protruberans: Google; Wikipedia; Merck; Images; PubMed (National Library of Medicine); NGC (National Guideline Clearinghouse); Medscape (eMedicine); Harrison's Online (accessmedicine); NEJM (The New England Journal of Medicine)

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