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Published in: Child's Nervous System 8/2021

Open Access 01-08-2021 | Ependymoma | Review Article

Pediatric ependymoma: an overview of a complex disease

Authors: Stephanie Theresa Jünger, Beate Timmermann, Torsten Pietsch

Published in: Child's Nervous System | Issue 8/2021

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Abstract

Pediatric ependymomas comprise biologically distinct tumor entities with different (epi)genetics, age distribution and localization, as well as a different prognosis. Regarding risk stratification within these biologically defined entities, histopathological features still seem to be relevant. The mainstay of treatment is gross total resection (GTR) if possible, achieved with intraoperative monitoring and neuronavigation—and if necessary second surgery—followed by adjuvant radiation therapy. However, there is growing evidence that some ependymal tumors may be cured by surgery alone, while others relapse despite adjuvant treatment. To date, the role of chemotherapy is not clear. Current therapy achieves reasonable survival rates for the majority of ependymoma patients. The next challenge is to go beyond initial tumor control and use risk-adapted therapy to reduce secondary effect and therapy-induced morbidity for low-risk patients and to intensify treatment for high-risk patients. With identification of specific alterations, targeted therapy may represent an option for individualized treatment modalities in the future.
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Metadata
Title
Pediatric ependymoma: an overview of a complex disease
Authors
Stephanie Theresa Jünger
Beate Timmermann
Torsten Pietsch
Publication date
01-08-2021
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 8/2021
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-021-05207-7

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