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J Neurooncol. 2017 Oct;135(1):201-211. doi: 10.1007/s11060-017-2568-8. Epub 2017 Jul 21.

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

Author information

1
Pediatric Neuro-Oncology Program, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Dana-Farber Cancer Institute and Boston Children's Hospital and Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA.
2
Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, MA, USA.
3
Departments of Pathology and Neurology, New York University Langone Medical Center and Medical School Pathology, New York, NY, USA.
4
Department of Neurology, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, 02215, MA, USA.
5
Department of Neurosurgery, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, 02215, MA, USA.
6
Pediatric Neuro-Oncology Program, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA. jfangusaro@luriechildrens.org.
7
Pediatric Neuro-Oncology Program, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Dana-Farber Cancer Institute and Boston Children's Hospital and Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA. Pratiti_Bandopadhayay@dfci.harvard.edu.

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.

KEYWORDS:

Ependymoma; Grade; Outcome; Resection; Survival

PMID:
28733870
PMCID:
PMC5658456
DOI:
10.1007/s11060-017-2568-8
[Indexed for MEDLINE]
Free PMC Article

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