Long-term survival and cure fraction estimates for paediatric central nervous system tumours in 31 European countries (EUROCARE-6): a population-based study

Abstract

Background: Clinically relevant survival outcomes, including cure fraction estimates, and long-term survival outcomes of paediatric CNS tumours from large-scale databases have not been reported for Europe. Moreover, various biases hinder direct geographical comparisons, thereby limiting the effective translation of population-based findings into cancer care, surveillance, and research. We aimed to estimate these survival outcomes across Europe through the EUROCARE database.

Methods: In this population-based study, we analysed survival data from the EUROCARE-6 database from children younger than 15 years with a CNS tumour across 31 European countries. For the period 2008-13, we estimated observed survival via the actuarial method, and 5-year observed survival was reported at the European level and national level for four major CNS tumour groups. For the period 1998-2013, cure fraction was estimated through a mixture cure model assuming constant long-term mortality from other causes. Additionally, model-based 10-year and 15-year survival were estimated.

Findings: For observed survival analyses, 13 782 tumour cases were included. 5-year observed survival was 72% (95% CI 68 to 75) for ependymomas, 92% (91 to 93) for low-grade gliomas, 47% (45 to 49) for high-grade gliomas, 24% (21 to 27) for high-grade gliomas excluding glioma not otherwise specified, and 64% (62 to 67) for medulloblastomas. A total of 30 392 children were included in the cure fraction analysis. During the study period, the largest absolute increase in cure fraction was observed for ependymomas from 65% (57 to 73) in 1998-2001 to 79% (69 to 89) in 2010-13, whereas low-grade gliomas increased from from 89% (85 to 94) to 95% (89 to 100), high-grade gliomas had a 6 percentage point change increase (2 to 10), and medulloblastomas increased from 52% (49 to 55) to 56% (51 to 60). The estimated 10-year and 15-year survival rates were highest for low-grade gliomas at 90·6% (89·4 to 91·7) at 10 years and 88·5% (87·2 to 89·8) at 15 years, whereas the lowest survival rates were observed for high-grade gliomas excluding glioma not otherwise specified at 20·5% (17·0 to 24·1) and 19·0% (15·6 to 22·5).

Interpretation: This study is the first to report a comprehensive evaluation of survival parameters for paediatric CNS tumour patients in Europe. These outcomes are important to evaluate advances in care for children with a CNS tumour.

Funding: Princess Máxima Center for Pediatric Oncology and Associazione Italiana per la Ricerca sul Cancro.