Denys-Drash syndrome



In this section a small Wilms tumor showing the 3 components is seen. Denys-Drash syndrome, which is associated

with a mutation in the WT1 gene at 11p13, is related to WAGR syndrome, which shows a deletion in that gene. Patients

with WAGR, Denys-Drash, or Beckwith-Wiedemann syndrome carry a high risk for Wilms tumors.


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