4.4 Article

Staging childhood cancers in Europe: Application of the Toronto stage principles for neuroblastoma and Wilms tumour. The JARC pilot study

Journal

PEDIATRIC BLOOD & CANCER
Volume 68, Issue 9, Pages -

Publisher

WILEY
DOI: 10.1002/pbc.29020

Keywords

Europe; neuroblastoma; population cancer registries; stage at diagnosis; survival; Wilms tumour

Funding

  1. European Union's Health Programme (2014-2020) [724161/JARC]

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The European Rare Cancers Joint Action conducted a pilot study on childhood cancer staging based on the 'Toronto consensus principles and guidelines'. The study found that around half of children diagnosed had localized or locoregional disease, with older age being associated with more advanced stage. Results indicated the clear prognostic value of stage information in investigating survival variations between countries or over time.
Background The 'Toronto consensus principles and guidelines' (TG) provided paediatric-specific staging system affordable by population-based cancer registries (CRs). Within the European Rare Cancers Joint Action, a pilot study of the application of TG for childhood cancer (CC) was conducted to test the ability of CRs to reconstruct stage, describe stage across countries and assess survival by stage. Procedure Twenty-five CRs representing 15 countries contributed data on a representative sample of patients with neuroblastoma (NB) and Wilms tumour (WT) <15 years, diagnosed between 2000 and 2016. Outcome was calculated by Kaplan-Meier method and by Cox regression model. Results Stage was reconstructed for 95% of cases. Around half of the children had localised or locoregional disease at diagnosis. The proportion of metastatic cases was 38% for NB and 13% for WT. Three-year survival was >90% for locoregional cases both of NB and WT, 58% for NB M-stage and 77% for WT stage-IV. Older age was associated with more advanced stage. Conclusions European CRs were able to reconstruct stage according to the TG. Stage should be included in the routine collection of variables. Stage information had clear prognostic value and should be used to investigate survival variations between countries or over time.

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