Wilms Tumour (Robbins pp 487, Fig 11-27/8)

General: Wilms tumour is renal cancer of childhood.

Epidemiology: 2-5 age group.

Pathogenesis/Genetics/Aetiology: The risk of Wilms tumour increases in association with 3 congenital malformations:

1. WAGR syndrome (WT-1 gene deleted),
   
2. Denys-Drash syndrome (WT-1 gene mutation),
   
3. Beck-with-Wiedemann syndrome (WT-2 abnormal).

Morphology: A risk factor for Wilms tumour is: nephroblastomatosis (i.e.: nephrogenic elements in kidney). Microscopy shows triphasic histology: stromal area (less cellular, spindle shaped cells), epithelial area, blastemic (tightly packed basophilic cells).

Clinical features: palpable mass, haematuria, costovertebral pain.

Prognosis / Treatment: Prognosis was poor in the 60s. Modern treatment of: chemotherapy, radiotherapy, surgery has lifted 5-year survival rate to 90%.

Category: Pathology Notes