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Desmoplastic small round cell tumor

Disease definition

Desmoplastic small round cell tumor (DSRCT) is 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.

ORPHA:83469

  • 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

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