Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor
- 12 May 2006
- journal article
- research article
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 47 (3), 260-267
- https://doi.org/10.1002/pbc.20891
Abstract
Background The National Wilms Tumor Study (NWTS) constitutes a unique resource for study of clinical, pathologic, and epidemiologic features of Wilms tumor (WT). Procedure Data from NWTS‐3,4,5 were compiled for 7,455 patients with tumors of favorable (FH) or anaplastic (AH) histology. The associations of birth weight (BW) and age‐at‐onset with gender, intralobar (ILNR), and perilobar (PLNR) nephrogenic rests, tumor focality, congenital malformation syndromes, and tumor histology were analyzed using descriptive statistics and linear regression. Results Mean BWs for male and female patients without PLNR were 3.52 and 3.36 kg, respectively, and for those with PLNR were 0.12 kg and 0.15 kg heavier. Mean age was 45 months for males with no rests whose tumors were unifocal and of triphasic favorable histology. ILNR or multifocality decreased the mean age by 18 and 10 months, respectively, whereas female gender, blastemal/FH or AH increased it by 3, 10, and 16 months. Over 90% of multifocal tumors occurred in the presence of demonstrated ILNR or PLNR or both. The apparent bimodality of the age distributions and later mean ages‐at‐onset for females with both unifocal and multifocal tumors were explained in part by the relative deficit in females of ILNR versus PLNR‐associated tumors. Conclusions These observations support the view that there are multiple pathways to Wilms tumorigenesis. They will facilitate selection of informative subgroups of patients for molecular analysis that may serve to identify the putative pathway for the majority of patients who cannot be classified provisionally on the basis of ILNR or PLNR. Pediatr Blood Cancer 2006;47:260–267.Keywords
This publication has 26 references indexed in Scilit:
- Children with Idiopathic Hemihypertrophy and Beckwith-Wiedemann Syndrome Have Different Constitutional Epigenotypes Associated with Wilms TumorAmerican Journal of Human Genetics, 2005
- Frequency and Heritability of WT1 Mutations in Nonsyndromic Wilms' Tumor Patients: A UK Children’s Cancer Study Group StudyJournal of Clinical Oncology, 2004
- Twenty‐four new cases of WT1 germline mutations and review of the literature: Genotype/phenotype correlations for Wilms tumor developmentAmerican Journal of Medical Genetics Part A, 2004
- Molecular Genetic Pathways to Wilms TumorCritical Reviews™ in Oncogenesis, 1997
- Relaxation of imprinted genes in human cancerNature, 1993
- Relaxation of insulin-like growth factor II gene imprinting implicated in Wilms' tumourNature, 1993
- Epidemiology of Wilms tumorMedical and Pediatric Oncology, 1993
- Introduction to the genetics of primary renal tumors in childrenMedical and Pediatric Oncology, 1993
- Nephrogenic Rests, Nephroblastomatosis, and the Pathogenesis of Wilms' TumorPediatric Pathology, 1990
- Mutation and Cancer: Statistical Study of RetinoblastomaProceedings of the National Academy of Sciences of the United States of America, 1971