PLoS ONE (Jan 2019)

Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

  • J A Hol,
  • M I Lopez-Yurda,
  • H Van Tinteren,
  • M Van Grotel,
  • J Godzinski,
  • G Vujanic,
  • F Oldenburger,
  • B De Camargo,
  • G L Ramírez-Villar,
  • C Bergeron,
  • K Pritchard-Jones,
  • N Graf,
  • M M Van den Heuvel-Eibrink

DOI
https://doi.org/10.1371/journal.pone.0221373
Journal volume & issue
Vol. 14, no. 8
p. e0221373

Abstract

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BackgroundTo enhance risk stratification for Wilms tumour (WT) in a pre-operative chemotherapy setting, we explored the prognostic significance and optimal age cutoffs in patients treated according to International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) protocols.MethodsPatients(6 months-18 years) with unilateral WT were selected from prospective SIOP 93-01 and 2001 studies(1993-2016). Martingale residual analysis was used to explore optimal age cutoffs. Outcome according to age was analyzed by uni- and multivariable analysis, adjusted for sex, biopsy(yes/no), stage, histology and tumour volume at surgery.Results5631 patients were included; median age was 3.4 years(IQR: 2-5.1). Estimated 5-year event-free survival (EFS) and overall survival (OS) were 85%(95%CI 83.5-85.5) and 93%(95%CI 92.0-93.4). Martingale residual plots detected no optimal age cutoffs. Multivariable analysis showed lower EFS with increasing age(linear trend PConclusionAlthough optimal age cutoffs could not be identified, we demonstrated the prognostic significance of age as well as previously described cutoffs for EFS (2 and 4 years) and OS (4 years) in children with WT treated with pre-operative chemotherapy. These findings encourage the consideration of age in the design of future SIOP-RTSG protocols.