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Neurocognitive, academic and functional outcomes in survivors of infant ependymoma (UKCCSG CNS 9204)

Abstract

Purpose

This is the first UK multi-centre case-controlled study with follow-up in excess of 10 years to report the neurocognitive, academic and psychological outcomes of individuals diagnosed with a brain tumour in early childhood. Children enrolled into the UKCCSG CNS 9204 trial, diagnosed with intracranial ependymoma when aged ≤ 36 months old, who received a primary chemotherapy strategy to defer or avoid radiotherapy, were recruited.

Methods

Outcomes of those who relapsed and subsequently received radiotherapy (n = 13) were compared to those enrolled who did not relapse (n = 16), age-matched controls—diagnosed with solid non-central nervous system (SN-CNS; n = 15) tumours or low-grade posterior fossa pilocytic astrocytoma (PFPA; n = 15), and normative data. Analyses compared nine neurocognitive outcomes as primary measures with quality of survival as secondary measures.

Results

Relapsed ependymoma participants performed significantly worse than their non-relapsed counterparts on measures of Full Scale IQ, Perceptual Reasoning, Word Reading and Numerical Operations. The relapsed ependymoma group performed significantly worse than SN-CNS controls on all primary measures, whereas non-relapsing participants only differed significantly from SN-CNS controls on measures of Processing Speed and General Memory. Relapsed ependymoma participants fared worse than all groups on measures of quality of survival.

Conclusions

The relapsed irradiated ependymoma group demonstrated the most significantly impaired neurocognitive outcomes at long-term follow-up. Non-relapsing participants demonstrated better outcomes than those who relapsed. Results tentatively suggest avoiding radiotherapy helped preserve neurocognitive and learning outcomes of individuals diagnosed with ependymoma when aged ≤ 36 months old. Prospective neurocognitive surveillance is required. Recommendations for clinical and research practice are provided.

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Acknowledgements

The authors would like to give their full thanks to all the participants and their families who consented to take part in this study, Candlelighters, Principal Investigators, Research Nurses, Data Managers, Trial Administrators, Dr. Bull, Prof. Kennedy, Mr. McShane and Dr. Phillips.

Funding

Candlelighters-Childhood cancer charity in Yorkshire provided funding. The sponsor had no role in the study design, conduct, data collection, data management, analysis, interpretation, preparation, review or approval of the report.

Author information

Correspondence to Matthew C. H. J. Morrall.

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Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee at which the studies were conducted. Ethical approval was awarded by the National Research Ethical Service (08/H1311/92).

Informed consent

Informed consent was obtained from all parents/guardians of participants, along with each participant providing informed assent.

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The authors declare that there are no conflicts of interest.

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Morrall, M.C.H.J., Reed-Berendt, R., Moss, K. et al. Neurocognitive, academic and functional outcomes in survivors of infant ependymoma (UKCCSG CNS 9204). Childs Nerv Syst 35, 411–420 (2019). https://doi.org/10.1007/s00381-018-4015-3

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Keywords

  • Brain tumour
  • Ependymoma
  • Paediatric
  • Outcome
  • Neurocognitive
  • Quality of survival