Wilms' tumour (WT) is an embryonal neoplasm arising in the kidney. Classical 'triphasic' WT has stromal, blastemal, and epithelial elements. the bone-metastasizing renal tumour (clear cell sarcoma) and malignant rhabdoid tumour are pathologically and genetically distinct entities, with their own clinical courses.
WT represents about 8% of all childhood neoplasia. The peak age for diagnosis is 3-4 years of age. It is very rare after the age of 10 years, but is occasionally diagnosed in adults.
There are a number of conditions known to predispose to the development of WT:
♦ Genitourinary abnormalities
♦ Beckwith-Wiedeman syndrome
♦ Denys-Drash syndrome
♦ Perlmann syndrome
♦ Simpson-Golabi-Behmel syndrome
This group of patients represents only a small proportion of the total number seen however.
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