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Age distribution of Wilms' tumor: report from the National Wilms' Tumor Study
N Breslow1, J B Beckwith, M Ciol
1Department of Biostatistics, University of Washington, Seattle 98195.
Insights
Wilms
Area of Science:
- Pediatric Oncology
- Cancer Genetics
- Developmental Biology
Background:
- Wilms' tumor is a pediatric kidney cancer with varying clinical presentations.
- Understanding its pathogenesis is crucial for diagnosis and genetic counseling.
- Previous models, like Knudson and Strong's, proposed a two-stage mutation process.
Purpose of the Study:
- To investigate the heterogeneity in Wilms' tumor pathogenesis.
- To analyze age at diagnosis in relation to tumor laterality, associated anomalies, and precursor lesions.
- To evaluate the implications for existing genetic models of Wilms' tumor.
Main Methods:
- Retrospective analysis of patient data including age at diagnosis, tumor characteristics, and associated conditions.
- Comparison of median ages at diagnosis across different subgroups.
- Correlation of precursor lesions (nephroblastomatosis) with disease presentation.
Main Results:
- Median age at diagnosis varied significantly based on laterality (unilateral vs. bilateral) and presence of associated anomalies (hemihypertrophy, aniridia, genitourinary anomalies).
- Patients with perilobar nephroblastomatosis were diagnosed later than those with intralobar nephroblastomatosis.
- Bilateral Wilms' tumor was frequently associated with precursor lesions.
Conclusions:
- Findings suggest significant heterogeneity in Wilms' tumor development.
- Age at diagnosis and associated conditions provide insights into distinct pathogenic pathways.
- The observed heterogeneity challenges certain aspects of the traditional two-stage mutation model for Wilms' tumor etiology.
Abstract:
Median ages at diagnosis were 36.5 and 42.5 mo for 1523 males and 1678 females with unilateral Wilms' tumor registered between October 1969 and December 1985; they were 23.5 mo and 30.5 mo for 100 males and 141 females with bilateral disease. The median age for multicentric, unilateral cases was intermediate between the bilateral and unicentric medians. Patients with hemihypertrophy in addition to their Wilms' tumor had a typical age distribution, whereas those with aniridia or characteristic genitourinary anomalies were substantially younger. Patients with perilobar nephroblastomatosis had a median age of 35.5 mo and those with intralobar nephroblastomatosis, a median age of 18.5 mo. Most of the bilateral disease occurred in the presence of one or both of these precursor lesions. These findings suggest heterogeneity in the pathogenesis of Wilms' tumor, having implications for genetic counselling, and call into question certain aspects of Knudson and Strong's two-stage mutational model for the origin of Wilms' tumor.