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Child's Nervous System

, Volume 35, Issue 12, pp 2327–2338 | Cite as

Childhood medulloblastoma—a single institution’s historical perspective on survival and functional morbidity

  • Kim Phipps
  • Matthew A. Kirkman
  • Kristian Aquilina
  • Mark Gaze
  • Antony Michalski
  • Angie Wade
  • Richard HaywardEmail author
Original Article
  • 38 Downloads

Abstract

Purpose

To compare results from a third (1995–2010) cohort of children with medulloblastoma with two previous series (J Neurosurg 86:13–21, 1997; Arch Dis Child 54:200–203, 1979) to analyse the effects of management changes aimed at improving both overall and event-free survivals (OS and EFS) and functional outcomes.

Methods

Review of neuro-oncology and imaging databases and previously published results.

Results

There was no statistically significant improvement in the 5-year OS for 104 children diagnosed 1995–2010, 61.5% (95% CI, 52.9, 71.6), compared with 50% of the 80 children presenting 1980–1990 (J Neurosurg 86:13–21, 1997) (difference 11.5%; 95% CI, 2.8, 25.4). Five-year OS for 96 children suitable for risk-stratification was overall 66% (95% CI, 57.9, 75.8); standard risk 77.8% (95% CI, 67.4, 89.7); high risk < 3 years 50.0% (95% CI, 32.3, 77.5); high risk ≥ 3 years 54.5% (95% CI, 37.2, 79.9); 5-year EFS were standard risk 68.5% (95% CI, 57.2, 82.1); high risk < 3 years 40.0% (95% CI, 23.4, 68.4); and high risk ≥ 3 years 36.4% (95% CI, 20.9, 63.2); overall 55.2% (95% CI, 46.1, 66.1). Of 62/63 ≥ 5-year survivor, 9 died later from tumour relapse and 4 from second malignancy. Functional outcomes of 62 of the 63 ≥ 5-year survivors: 67.7% had educational issues requiring remedial input; 18% restricted mobility indoors and outdoors; 59.7% hearing impairment (42% prescribed aids).

Conclusions

  1. 1.

    Comparison of this single-institution series with its predecessor found that revised chemotherapy and RT protocols and greater accuracy of risk stratification did not result in statistically significant improvements in either survival or treatment-related functional disability.

     
  2. 2.

    Extended (> 5-year) follow-up is essential if 20% of late deaths from relapse and second malignancies are not to be overlooked.

     

Keywords

Medulloblastoma Radiotherapy Neurosurgery,  Chemotherapy,  Functional outcome 

Notes

Funding information

MNG is supported by the National Institute for Health Research, University College London Hospitals Biomedical Research Centre, London, UK.

Compliance with Ethical Standards

Conflict of Interest

The authors have declared no conflicts of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Kim Phipps
    • 1
  • Matthew A. Kirkman
    • 1
    • 2
  • Kristian Aquilina
    • 1
  • Mark Gaze
    • 3
    • 4
  • Antony Michalski
    • 3
  • Angie Wade
    • 5
  • Richard Hayward
    • 1
    Email author
  1. 1.Department of NeurosurgeryGreat Ormond Street Hospital for Children NHS Foundation TrustLondonUK
  2. 2.Victor Horsley Department of NeurosurgeryThe National Hospital for Neurology and NeurosurgeryLondonUK
  3. 3.Department of Haematology and OncologyGreat Ormond Street Hospital for Children NHS Foundation TrustLondonUK
  4. 4.Department of OncologyUniversity College London Hospitals NHS Foundation TrustLondonUK
  5. 5.Population, Policy and Practice ProgrammeGreat Ormond Street Institute of Child Health, UCLLondonUK

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