Incidence of cardiac septal defects in children with Wilms' tumour and other malignant diseases.

In a population-based series of 8045 children with malignant neoplasms in Great Britain, the incidence of septal defects was 0.40%. The high rate of 19% in Down's syndrome children and the overall rate of 0.28% in children without Down's syndrome were both comparable with rates found in previous large prospective studies. The incidence of septal defects in children with Wilms' tumour was 1.82%, greater than 10 times that for non-Down's children with other neoplasms. The presence of this association in Wilms' tumour patients with and without aniridia and the recent finding of a loss of heterozygosity in chromosome 11 in many Wilms' tumours taken from patients without aniridia suggest the possibility of a link between Wilms' tumour, some septal defects and chromosomal abnormalities.