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Incidence and survival for childhood central nervous system tumours in Australia, 1983–2016

Abstract

Purpose

To investigate incidence and survival of childhood tumours of the central nervous system (CNS) by histological subtype, tumour behaviour and tumour grade.

Methods

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.

Results

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.

Conclusion

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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Data availability

Access to de-identified unit record data from the Australian Childhood Cancer Registry is limited to named investigators on the study protocol.

Code availability

All programs used to generate the results are available on request.

Abbreviations

ACCR:

Australian Childhood Cancer Registry

APC:

Annual percentage change

CI:

Confidence interval

CNS:

Central nervous system

HR:

Hazard ratio

ICCC-3:

International classification of childhood cancers, version 3

ICD-O:

International classification of diseases for oncology

WHO:

World Health Organisation

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Acknowledgements

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.

Funding

Nil.

Author information

Affiliations

Authors

Contributions

DY, JA, NG and TH developed the study concept and design. Material preparation, data collection and analyses were performed by CH and DY. The first draft of the manuscript was written by DY and CH and all authors commented on and contributed to further versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Danny R. Youlden.

Ethics declarations

Conflict of interest

The authors have no competing interests to disclose.

Ethical approval

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).

Consent to participate

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.

Consent for publication

Not applicable.

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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

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Keywords

  • Childhood cancer
  • Central nervous system
  • Incidence
  • Survival
  • Australia
  • Population-based registry