Epidemiological features of Wilms' tumor: Results of studies by the international society of paediatric oncology (SIOP)

Abstract

This descriptive epidemiology study of 1,040 children with Wilms' tumor (WT) registered in the International Society of Paediatric Oncology (SIOP) clinical trials confirms the findings reported by the National Wilms' Tumor Study. The male:female rate was 0.89:1. The mean age at diagnosis of the 43 bilateral cases was significantly younger than children with unilateral renal involvement (32.4 vs 45 months). However, the mean ages of diagnosis for unilateral multicentric and for unicentric WT were very similar. On the other hand, the mean age at diagnosis of children with sporadic aniridia and hypospadias was younger than the mean age of patients with or without other congenital malformations. Thus aniridia as well as hypospadias could be indices of the first mutation, according to the Knudson and Stron hypothesis. WT was reported in two members of each of five families. However, these familial cases were comparable in terms of demographic and clinical features to the nonfamilial ones. These data suggest that the heritable fraction of WT is relatively small and that genetic and environmental factors interact in the development of WT.

Epidemiological and clinical correlations with genetic characteristics of Wilms' tumor: Results of the Brazilian Wilms' tumor study group

“…The mean age at diagnosis among our bilateral cases was substantially lower than that for any other group of patients with unilateral WT. Occurrence of congenital defects in the GCBTTW study was within the expectation bounds of both the North American (Breslow and Beckwith, 1982) and the European (Pastore et al, 1988) rates. Unlike the findings in these studies, however, the mean age of our patients with congenital anomalies was higher than that for the remaining children.…”

International variations in the incidence of childhood renal tumours

“…These include bilateral tumours, those which occur in association with aniridia and certain other congenital abnormalities, and the small number of cases which form part of familial aggregations. Fewer than one in 15 cases of Wilms' tumour are bilateral and the other classes of 'genetic' Wilms' tumour account for even smaller proportions (Breslow & Beckwith, 1982;Pastore et al, 1988). The frequency of associated congenital abnormalities is higher in Blacks, who have a higher incidence of Wilms' tumour, than in Whites (Kramer et al, 1984).…”

Childhood Acute Leukemias in Hispanic Population: Differences by Age Peak and Immunophenotype

“…However, in the case of children born with the rearrangement ETV6/RUNX1, one or more additional risk factors (e.g., an exaggerated response to late common infections) would be needed to initiate leukemogenesis. It is possible that the age peak of ALL reflects a higher degree of susceptibility with which a child is born, such as is the case with both bilateral retinoblastoma and bilateral Wilm's tumor: an earlier age peak is found in the bilateral forms of these diseases than when the retinoblastoma or Wilm's tumor is unilateral (National Wilm's Tumor Study Committee, 1999;Pastore et al, 1988;Sanders et al, 1988;Teppo et al, 1975). Thus, children with the highest susceptibility at birth should show an early age peak for ALL (say, during the firsts year of life), whereas children born with the lowest susceptibility will evidence an age peak of ALL in later years.…”