Posterior fossa ependymomas in children and adolescents - the state of the art
DOI:
https://doi.org/10.46900/apn.v5i3.210Keywords:
ependymoma, Posterior fossa tumor, Pediatric neurosurgery, neuro-oncologyAbstract
Introduction: Central nervous system tumors in pediatric patients represent the most frequent solid tumor type in this age group, especially ependymomas, which comprise 5% of childhood intracranial tumors and occur mainly in the posterior fossa.
Methods: The MEDLINE and ClinicalTrials databases were searched for studies on posterior fossa ependymomas, 64 studies met the search criteria.
Results: Symptoms include ataxia, vertigo, headache, vomiting, cranial nerve palsies, or papilledema resulting from intracranial hypertension due to obstruction of CSF circulation. Imaging examination often reveals posterior fossa ependymomas in the fourth ventricle or cerebellopontine angle; they may extend through the foramina of Luschka, Magendie, and/or magnum. These tumors cause a significant mass effect, displacing rather than invading the hidbrain, and may involve vessels or cranial nerves, making surgical resection difficult. Various radiological features have been studied to distinguish between ependymomas and other histological types, as well as between ependymoma subtypes.
The 2021 WHO classification of central nervous system tumors classifies ependymomas by location. Three types occur in the posterior fossa: subependymomas, PF-A, and PF-B. These are differentiated by the degree of DNA methylation and CpG islands, which can be assessed by immunohistochemical analysis with H3K27me3.
Conclusion: The consensus on the treatment of PF ependymomas in children advocates the maximum possible surgical resection, followed by radiotherapy in patients >1 year. Chemotherapy remains controversial. Recent studies suggest molecular subgroups as an independent prognostic factor in posterior fossa ependymomas. The therapeutic challenge of ependymomas requires new treatment options, based on new technologies and molecular discoveries.
Downloads
Additional Files
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2023 Marcos Devanir Silva da Costa, Carolina Torres Soares, Patricia Alessandra Dastoli, Jardel Mendonça Nicácio, Frederico Adolfo Benevides Silva, Maria Teresa de Seixas Alves, Michael Jenwei Chen, Andrea Maria Cappellano, Nasjla Saba Silva, Sergio Cavalheiro
This work is licensed under a Creative Commons Attribution 4.0 International License.
When publishing in Archives of Pediatric Neurosurgery journal, authors retain the copyright of their article and agree to license their work using a Creative Commons Attribution 4.0 International Public License (CC BY 4.0), thereby accepting the terms and conditions of this license (https://creativecommons.org/licenses/by/4.0/legalcode).
The CC BY 4.0 license terms applies to both readers and the publisher and allows them to: share (copy and redistribute in any medium or format) and adapt (remix, transform, and build upon) the article for any purpose, even commercially, provided that appropriate credit is given to the authors and the journal in which the article was published.
Authors grant Archives of Pediatric Neurosurgery the right to first publish the article and identify itself as the original publisher. Under the terms of the CC BY 4.0 license, authors allow the journal to distribute the article in third party databases, as long as its original authors and citation details are identified.
