Orphanet: Desmoplastic small round cell tumor

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Desmoplastic small round cell tumor

Disease definition

An aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases.


Classification level: Disorder
  • Synonym(s):
    • DSRCT
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adolescent, Adult
  • ICD-10: C48.2
  • OMIM: -
  • UMLS: C0281508
  • MeSH: D058405
  • GARD: 6265
  • MedDRA: 10064581

Detailed information

Article for general public


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