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Open Access 01-10-2017 | Clinical Study

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

Authors: Amanda E. Marinoff, Clement Ma, Dongjing Guo, Matija Snuderl, Karen D. Wright, Peter E. Manley, Hasan Al-Sayegh, Claire E. Sinai, Nicole J. Ullrich, Karen Marcus, Daphne Haas-Kogan, Liliana Goumnerova, Wendy B. London, Mark W. Kieran, Susan N. Chi, Jason Fangusaro, Pratiti Bandopadhayay

Published in: Journal of Neuro-Oncology | Issue 1/2017

Abstract

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.

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Title: Rethinking childhood ependymoma: a retrospective, multi-center analysis reveals poor long-term overall survival
Authors: Amanda E. Marinoff
Clement Ma
Dongjing Guo
Matija Snuderl
Karen D. Wright
Peter E. Manley
Hasan Al-Sayegh
Claire E. Sinai
Nicole J. Ullrich
Karen Marcus
Daphne Haas-Kogan
Liliana Goumnerova
Wendy B. London
Mark W. Kieran
Susan N. Chi
Jason Fangusaro
Pratiti Bandopadhayay
Publication date: 01-10-2017
Publisher: Springer US
Published in: Journal of Neuro-Oncology / Issue 1/2017
Print ISSN: 0167-594X
Electronic ISSN: 1573-7373
DOI: https://doi.org/10.1007/s11060-017-2568-8

At a glance: The STEP trials

Springer Medicine

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

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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