Orphanet: Dermatofibrosarcoma protuberans

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Dermatofibrosarcoma protuberans

Disease 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).


Classification level: Disorder
  • Synonym(s):
    • DFSP
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: C49.9
  • OMIM: 607907
  • UMLS: C0392784
  • MeSH: C538219
  • GARD: 9569
  • MedDRA: 10057070

Detailed information

Article for general public


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