ANAPLASTIC WILMS-TUMOR, A SUBTYPE DISPLAYING POOR-PROGNOSIS, HARBORS P53 GENE-MUTATIONS

The genetics of Wilms' tumour (WT), a paediatric malignancy of the kid ney, is complex. Inactivation of the tumour suppressor gene, WT1, is a ssociated with tumour aetiology in similar to 10-15% of WTs. Chromosom e 17p changes have been noted in cytogenetic studies of WTs, prompting us to screen 140 WTs for p53 mutations. When histopathology reports w ere available, p53 mutations were present in eight of eleven anaplasti c WTs, a tumour subtype associated with poor prognosis. Amplification of MDM2, a gene whose product binds and sequesters p53, was excluded. Our results indicate that p53 alterations provide a molecular marker f or anaplastic WTs.