To investigate incidence and survival of childhood tumours of the central nervous system (CNS) by histological subtype, tumour behaviour and tumour grade.
National, population-based data on all children under 15 years old diagnosed with a CNS tumour between 1983 and 2016 were sourced from the Australian Childhood Cancer Registry. Incidence rate trends were calculated using Joinpoint regression. Relative survival was calculated using the cohort method, with changes in survival over time by cancer type and tumour grade assessed by multivariable flexible parametric survival modelling.
The study cohort included 4914 patients, with astrocytoma (n = 2181, 44%) and embryonal tumours (n = 931, 19%) the most common diagnostic subgroups. Almost half (n = 2181, 44%) of all tumours were classified as high grade (III or IV). Incidence rates increased by 29% between 1983 and 2016, with high grade tumours rising by an annual average of + 1.1% (95% CI = + 0.7%, + 1.5%, p < 0.001). 5-year survival for all patients combined was 72% (95% CI = 71–74%), ranging from 50% (46–54%) for those with other gliomas to 81% (79–83%) for astrocytoma (p < 0.001). Survival improved over time for grade II and III ependymomas but not for patients with astrocytoma irrespective of grade.
Improvements in diagnostic technology leading to more precise tumour classification are likely to explain some of the differences in incidence rate trends by histological type and grade. While improvements in survival over time were noted for some tumours, outcomes remained poor among patients with high-grade astrocytoma.
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Access to de-identified unit record data from the Australian Childhood Cancer Registry is limited to named investigators on the study protocol.
All programs used to generate the results are available on request.
Australian Childhood Cancer Registry
Annual percentage change
Central nervous system
International classification of childhood cancers, version 3
International classification of diseases for oncology
World Health Organisation
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The authors wish to thank Leisa O’Neill and Leanne Keeley for their work in the Australian Childhood Cancer Registry. We also acknowledge the assistance of staff at all Australian State and Territory Cancer Registries, the Australian Institute of Health and Welfare and the Medical Records Department at each of the major paediatric oncology treating hospitals throughout Australia.
Conflict of interest
The authors have no competing interests to disclose.
Approval for the ongoing operation of the Australian Childhood Cancer Registry is obtained from the University of Queensland Behavioural and Social Sciences Ethical Review Committee (reference number 2004000302).
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A waiver of informed consent has been approved for the ACCR. There is strictly no contact made with any individual included in the study. No information is released by which it would be possible to identify an individual person.
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Youlden, D.R., Henshaw, C., Gottardo, N.G. et al. Incidence and survival for childhood central nervous system tumours in Australia, 1983–2016. J Neurooncol 155, 203–213 (2021). https://doi.org/10.1007/s11060-021-03869-6
- Childhood cancer
- Central nervous system
- Population-based registry