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Neuro Oncol. 2019 Mar 18;21(4):547-557. doi: 10.1093/neuonc/noy191.

Craniospinal irradiation as part of re-irradiation for children with recurrent intracranial ependymoma.

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Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, Toronto, Canada.
Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Canada.
Radiotherapy Research Group, University of Leeds, Leeds, United Kingdom.
Department of Pediatric Haematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic.
Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto, Canada.
Division of Neurosurgery, Hospital for Sick Children, Toronto, Canada.



The goal of this study was to evaluate outcomes in children with relapsed, molecularly characterized intracranial ependymoma treated with or without craniospinal irradiation (CSI) as part of a course of repeat radiation therapy (re-RT).


This was a retrospective cohort study of 31 children. Patients with distant relapse received CSI as part of re-RT. For patients with locally recurrent ependymoma, those treated before 2012 were re-irradiated with focal re-RT. In 2012, institutional practice changed to offer CSI, followed by boost re-RT to the site of resected or gross disease.


Median follow-up was 5.5 years. Of 9 patients with distant relapse after initial RT, 2-year freedom from progression (FFP) and overall survival (OS) were 12.5% and 62.5%, respectively. There were 22 patients with local failure after initial RT. In these patients, use of CSI during re-RT was associated with improvement in 5-year FFP (83.3% with CSI vs 15.2% with focal re-RT only, P = 0.030). In the subgroup of patients with infratentorial primary disease, CSI during re-RT also improved 5-year FFP (100% with CSI, 10.0% with focal re-RT only, P = 0.036). Twenty-three patients had known molecular status; all had posterior fossa group A tumors (n = 17) or tumors with a RELA (v-rel avian reticuloendotheliosis viral oncogene homolog A) fusion (n = 6). No patient developed radiation necrosis after fractionated re-RT, though almost all survivors required assistance throughout formal schooling. Five out of 10 long-term survivors have not developed neuroendocrine deficits.


Re-irradiation with CSI is a safe and effective treatment for children with locally recurrent ependymoma and improves disease control compared with focal re-irradiation, with the benefit most apparent for those with infratentorial primary tumors.


ependymoma; pediatrics; re-irradiation; recurrence

[Available on 2020-03-18]
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