Molecular genetics of Wilms' tumour.

Wilms' tumour, or nephroblastoma, is an embryonal malignancy of the kidney with an incidence of approximately 1 in 10,000 live births. It occurs in both sporadic and familial forms, but only 1% of Wilms' tumour patients have a positive family history. The molecular genetics of Wilms' tumour have been the subject of extensive research and at least three genes (WT1, WT2, WT3) have been implicated. WT1 has been mapped to 11p13, and it has been suggested that loss or inactivation of a tumour-suppressor gene at 11p13 might be a primary event in the development of Wilms' tumour. The WT2 gene maps to 11p15 in the region of the Beckwith-Wiedemann locus. The WT3 locus is likely to be located to chromosome 16q. The understanding of the molecular genetics of Wilms' tumour is reviewed briefly.