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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.

Author information

1
Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, Toronto, Canada.
2
Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Canada.
3
Radiotherapy Research Group, University of Leeds, Leeds, United Kingdom.
4
Department of Pediatric Haematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic.
5
Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto, Canada.
6
Division of Neurosurgery, Hospital for Sick Children, Toronto, Canada.

Abstract

BACKGROUND:

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).

METHODS:

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.

RESULTS:

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.

CONCLUSIONS:

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.

KEYWORDS:

ependymoma; pediatrics; re-irradiation; recurrence

PMID:
30452715
PMCID:
PMC6422429
[Available on 2020-03-18]
DOI:
10.1093/neuonc/noy191
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