The most common kidney tumor in children.

  • Arises from metanephric precursor cells which form a tumor composed of blastemal, stromal, epithelial cells
  • Unknown etiology, some cases due to mutations of WT1 and WT2 genes
  • Part of the WAGR syndrome
    • Wilms tumor
    • Aniridia
    • Gonadal dysfunction
    • (Mental)

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