Wilms tumour and associated syndromes

Wilms tumour

  1. Also known as nephroblastoma.
  2. Wilms tumour is the most common primary renal tumour in children.
  3. Usually wilms tumour is a solitary,well circumscribed lesion,but 10% is bilateral or multicentric.
  4. Triphasic combination of epithelial,stromal and blastemal elements.
  5. 5% show anaplasia associated with underlying P53 mutation and unresponsive to chemotherapy.
  6. Nephrogenic cell rests- High correlation with Wilms tumour in contralateral kidney.

Syndromes associated with wilms tumour

WAGR syndrome

  1. Wilms tumour
  2. Aniridia
  3. Genital abnormalities
  4. Mental retardation

Denys Drash Syndrome

  1. Gonadal dysgenesis(Male pseudohermaphroditism)
  2. Renal abnormalities

Beckwith Weidman syndrome

  1. Organomegaly
  2. Hemihypertrophy
  3. Renal medullary cysts
  4. Hepatoblastoma
  5. Adrenal cortical tumour
  6. Rhabdomyosarcoma
  7. Pancreatic tumour

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