Rethinking childhood ependymoma: a retrospective, multi-center analysis reveals poor long-term overall survival

Ependymoma is the third most common brain tumor in children, but there is a paucity of large studies with more than 10 years of follow-up examining the long-term survival and recurrence patterns of this disease. We conducted a retrospective chart review of 103 pediatric patients with WHO Grades II/I...

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Bibliographic Details
Published in:Journal of neuro-oncology 2017-10, Vol.135 (1), p.201-211
Main Authors: Marinoff, Amanda E., Ma, Clement, Guo, Dongjing, Snuderl, Matija, Wright, Karen D., Manley, Peter E., Al-Sayegh, Hasan, Sinai, Claire E., Ullrich, Nicole J., Marcus, Karen, Haas-Kogan, Daphne, Goumnerova, Liliana, London, Wendy B., Kieran, Mark W., Chi, Susan N., Fangusaro, Jason, Bandopadhayay, Pratiti
Format: Article
Language:English
Subjects:
Adolescent
Brain Neoplasms - epidemiology
Brain Neoplasms - pathology
Brain Neoplasms - therapy
Brain tumors
Cancer
Child
Child, Preschool
Children
Clinical Study
Ependymoma - epidemiology
Ependymoma - pathology
Ependymoma - therapy
Female
Follow-Up Studies
Humans
Infant
Male
Medical prognosis
Medicine
Medicine & Public Health
Neoplasm Grading
Neoplasm Recurrence, Local - epidemiology
Neurology
Oncology
Retrospective Studies
SEER Program
Survival
Survival Analysis
Treatment Outcome
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Summary:Ependymoma is the third most common brain tumor in children, but there is a paucity of large studies with more than 10 years of follow-up examining the long-term survival and recurrence patterns of this disease. We conducted a retrospective chart review of 103 pediatric patients with WHO Grades II/III intracranial ependymoma, who were treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center and Chicago’s Ann & Robert H. Lurie Children’s Hospital between 1985 and 2008, and an additional 360 ependymoma patients identified from the Surveillance Epidemiology and End Results (SEER) database. For the institutional cohort, we evaluated clinical and histopathological prognostic factors of overall survival (OS) and progression-free survival (PFS) using the log-rank test, and univariate and multivariate Cox proportional-hazards models. Overall survival rates were compared to those of the SEER cohort. Median follow-up time was 11 years. Ten-year OS and PFS were 50 ± 5% and 29 ± 5%, respectively. Findings were validated in the independent SEER cohort, with 10-year OS rates of 52 ± 3%. GTR and grade II pathology were associated with significantly improved OS. However, GTR was not curative for all children. Ten-year OS for patients treated with a GTR was 61 ± 7% and PFS was 36 ± 6%. Pathological examination confirmed most recurrent tumors to be ependymoma, and 74% occurred at the primary tumor site. Current treatment paradigms are not sufficient to provide long-term cure for children with ependymoma. Our findings highlight the urgent need to develop novel treatment approaches for this devastating disease.
ISSN:0167-594X
1573-7373
DOI:10.1007/s11060-017-2568-8