PMID- 16700047 OWN - NLM STAT- MEDLINE DCOM- 20070109 LR - 20181113 IS - 1545-5009 (Print) IS - 1545-5017 (Electronic) IS - 1545-5009 (Linking) VI - 47 IP - 3 DP - 2006 Sep TI - Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor. PG - 260-7 AB - 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. FAU - Breslow, Norman E AU - Breslow NE AD - Department of Biostatistics, University of Washington, Seattle, Washington 98195-7232, USA. norm@u.washington.edu FAU - Beckwith, J Bruce AU - Beckwith JB FAU - Perlman, Elizabeth J AU - Perlman EJ FAU - Reeve, Anthony E AU - Reeve AE LA - eng GR - R01 CA054498/CA/NCI NIH HHS/United States GR - R01 CA54998/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Pediatr Blood Cancer JT - Pediatric blood & cancer JID - 101186624 SB - IM CIN - Pediatr Blood Cancer. 2006 Sep;47(3):232-4. PMID: 16830319 MH - Age Distribution MH - Age of Onset MH - Birth Weight MH - Child, Preschool MH - Female MH - Genetic Heterogeneity MH - Humans MH - Infant MH - Kidney Neoplasms/diagnosis/*genetics/*pathology MH - Male MH - Prognosis MH - Wilms Tumor/diagnosis/*genetics/*pathology PMC - PMC1543666 MID - NIHMS10538 EDAT- 2006/05/16 09:00 MHDA- 2007/01/11 09:00 PMCR- 2008/01/28 CRDT- 2006/05/16 09:00 PHST- 2006/05/16 09:00 [pubmed] PHST- 2007/01/11 09:00 [medline] PHST- 2006/05/16 09:00 [entrez] PHST- 2008/01/28 00:00 [pmc-release] AID - 10.1002/pbc.20891 [doi] PST - ppublish SO - Pediatr Blood Cancer. 2006 Sep;47(3):260-7. doi: 10.1002/pbc.20891.