Association of Chromosome 1q Gain With Inferior Survival in Favorable-Histology Wilms Tumor: A Report From the Children's Oncology Group

J Clin Oncol. 2016 Sep 10;34(26):3189-94. doi: 10.1200/JCO.2015.66.1140. Epub 2016 Jul 11.


Purpose: The goal of this study was to analyze the association of copy number gain of 1q in favorable-histology Wilms tumors (FHWTs) with event-free survival (EFS) and overall survival (OS) within each tumor stage and with 1p and 16q copy number loss and/or loss of heterozygosity.

Methods: Unilateral FHWTs from 1,114 patients enrolled in National Wilms Tumor Study-5 that were informative for 1p and 16q microsatellite markers (previously determined) and informative for 1q gain, 1p loss, and 16q loss using multiplex ligation-dependent probe amplification were analyzed.

Results: Eight-year EFS was 86% (95% CI, 84% to 88%) for the entire cohort. Of 1,114 patients, 317 tumors (28%) displayed 1q gain. Eight-year EFS was 77% for those with 1q gain and 90% for those lacking 1q gain (P < .001). Eight-year OS was 88% for those with 1q gain and 96% for those lacking 1q gain (P < .001). Within each disease stage, 1q gain was associated with inferior EFS (stage I, 85% v 95%; P = .0052; stage II, 81% v 87%; P = .0775; stage III, 79% v 89%; P = .01; stage IV, 64% v 91%; P = .001). OS was significantly inferior in patients with stage I (P < .0015) and stage IV disease (P = .011). With multivariable analysis, 1q gain was associated with an increased relative risk of relapse of 2.4 (P < .001), whereas 1p loss was not, despite significance on univariable analysis.

Conclusion: Gain of 1q is associated with inferior survival in unilateral FHWTs and may be used to guide risk stratification in future studies.

Publication types

  • Multicenter Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Biomarkers, Tumor / genetics*
  • Chi-Square Distribution
  • Child, Preschool
  • Chromosome Aberrations*
  • Chromosomes, Human, Pair 1*
  • DNA Copy Number Variations*
  • Disease Progression
  • Disease-Free Survival
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Multiplex Polymerase Chain Reaction
  • Multivariate Analysis
  • Neoplasm Recurrence, Local
  • Neoplasm Staging
  • North America
  • Phenotype
  • Predictive Value of Tests
  • Proportional Hazards Models
  • Prospective Studies
  • Risk Factors
  • Time Factors
  • Wilms Tumor / genetics*
  • Wilms Tumor / mortality
  • Wilms Tumor / pathology
  • Wilms Tumor / therapy


  • Biomarkers, Tumor