Relapsed intracranial ependymoma in children in the UK: Patterns of relapse, survival and therapeutic outcome

Messahel, B.; Ashley, S.; Saran, F.; Ellison, D.; Ironside, J.; Phipps, K.; Cox, T.; Chong, W.K.; Robinson, K.; Picton, S.; Pinkerton, C.R.; Mallucci, C.; Macarthur, D.; Jaspan, T.; Michalski, A.; Grundy, R.G.; On behalf of the Children’s Cancer Leukaemia Group Brain Tumour Committee,

doi:10.1016/j.ejca.2009.03.018

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Volume 45, Issue 10 , Pages 1815-1823, July 2009

Relapsed intracranial ependymoma in children in the UK: Patterns of relapse, survival and therapeutic outcome

B. Messahel
- Department of Paediatrics, Royal Marsden Hospital, Sutton, Surrey SM2 5PT, UK
S. Ashley
- Department of Paediatrics, Royal Marsden Hospital, Sutton, Surrey SM2 5PT, UK
F. Saran
- Department of Paediatrics, Royal Marsden Hospital, Sutton, Surrey SM2 5PT, UK
D. Ellison
- Department of Pathology, St. Jude Children’s Research Hospital, Memphis, TN 38105, USA
J. Ironside
- Western General Hospital NHS Trust, Edinburgh, EH4 2XU, UK
K. Phipps
- Great Ormond Street Children’s Hospital, Great Ormond Street, London WC1N 3JN, UK
T. Cox
- Great Ormond Street Children’s Hospital, Great Ormond Street, London WC1N 3JN, UK
W.K. Chong
- Great Ormond Street Children’s Hospital, Great Ormond Street, London WC1N 3JN, UK
K. Robinson
- Data Centre, UKCCSG, 9 Princess Road West, Leicester LE1, UK
S. Picton
- Regional Paediatric Oncology Unit, St. James University Hospital, Leeds LS9 7TF, UK
C.R. Pinkerton
- Director of Cancer Services, Mater Hospitals, Raymond Terrace, Brisbane OLD 4101, UK
C. Mallucci
- The Walton Centre for Neurosurgery, Liverpool L9 7LJ, UK
D. Macarthur
- The Children’s Brain Tumour Research Centre, University of Nottingham, The Medical School, Queen’s Medical Centre, Nottingham NG7 2UH, UK
T. Jaspan
- The Children’s Brain Tumour Research Centre, University of Nottingham, The Medical School, Queen’s Medical Centre, Nottingham NG7 2UH, UK
A. Michalski
- Great Ormond Street Children’s Hospital, Great Ormond Street, London WC1N 3JN, UK
R.G. Grundy
- The Children’s Brain Tumour Research Centre, University of Nottingham, The Medical School, Queen’s Medical Centre, Nottingham NG7 2UH, UK
- Corresponding author: Tel.: +44 (0)115 823 0620; fax: +44 (0)115 823 0696.
On behalf of the Children’s Cancer Leukaemia Group Brain Tumour Committee

Received 21 November 2008; received in revised form 8 March 2009; accepted 17 March 2009. published online 12 May 2009.

Abstract

Relapsed ependymoma in children poses difficult dilemmas in management. Clinico-pathological and treatment data of 108 children with relapsed ependymoma in the United Kingdom (UK) treated between 1985 and 2002 were reviewed to identify prognostic factors affecting survival. The primary site was the most common site of relapse (84%). Overall 25% had metastatic relapse. Surgery at relapse was attempted in only 55%. Radiotherapy was delivered at relapse in 66% infants and 50% of older children were re-irradiated. Overall 5-year survival was 24% and 27% for children less than 3years of age at initial diagnosis and older children, respectively. Multivariate analysis showed that, for infants, surgery (p=0.01) and radiotherapy (p=0.001) at relapse were independent predictors of survival. For older children regardless of the previous radiotherapy, repeat irradiation was associated with better outcome (p=0.05).

Relapse was associated with poor outcome in both age groups. A survival advantage conferred by both radiotherapy and surgery at relapse is independently significant.

Keywords: Ependymoma, Recurrence, Chemotherapy, Radiotherapy, Surgery, Survival