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GENATLAS PHENOTYPE
last update : 26/06/2006
Symbol DSRCT
Location 11p13 and 22q12.2
Name desmoplastic small round cell tumor
Corresponding gene WT1 , EWSR1
Main clinical features
  • primitive multiphenotypic sarcoma arising in serosal tissues
    • Genetic determination
    Function/system disorder neoplasia
    Type malignancy
    Gene product
    Name aberrant transcription factor,chimeric fusion protein 5'- EWS - WT1 -3'
    Mechanism(s)
    Gene mutationChromosome rearrangementEffectComments
      translocation   acquired t(11;22)(p13;p12) is the cytogenetic hallmark of DSRCT
    Remark(s)