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


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.

SUBMITTER: Tsang DS 

PROVIDER: S-EPMC6422429 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

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

Tsang Derek S DS   Murray Louise L   Ramaswamy Vijay V   Zapotocky Michal M   Tabori Uri U   Bartels Ute U   Huang Annie A   Dirks Peter B PB   Taylor Michael D MD   Hawkins Cynthia C   Bouffet Eric E   Laperriere Normand N  

Neuro-oncology 20190301 4


<h4>Background</h4>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).<h4>Methods</h4>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  ...[more]

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