Molecular genetics of Wilms' tumour

Wilms' tumour, or nephroblastoma, is an embryonal malignancy of the kidney with an incidence of approximately 1 in 10000 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 ( WT 1, WT 2, WT 3) have been implicated. WT 1 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 WT 2 gene maps to 11p15 in the region of the Beckwith-Wiedemann locus. The WT 3 locus is likely to be located to chromosome 16q. The understanding of the molecular genetics of Wilms' tumour is reviewed briefly.