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Review and update on pediatric ependymoma

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Abstract

Since our last Special Annual Issue dedicated to the topic of ependymoma in 2009, critical advancements have been made in the understanding of this disease which is largely confined to childhood. In the era of molecular profiling, the prior classification of ependymoma based on histology has become largely irrelevant, with multiple new subtypes of this disease now being described in the newest 2021 WHO CNS Tumor Classification System. Despite our advancements in understanding the underlying biology of these tumors, the mainstays of treatment—gross total surgical resection followed by confocal radiation therapy—have continued to yield the best treatment results across multiple studies and centers. Here, we provide an update on our understanding of the advancements made in tumor biology, surgical, and oncologic management of this disease. As we move into an era of more personalized medicine, it is critical to reflect on our historical understanding of different disease entities, to better understand the future directions of our treatments.

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Data Availability

The authors confirm that the data presented is based upon synthesis of published references and not unpublished or personal datasets. Specific data referenced within may be found on publishers’ websites for the respective references.

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Authors and Affiliations

  1. Department of Neurological Surgery, University of Washington, Seattle, WA, USA

    Scott H. Boop

  2. Department of Surgery, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Nir Shimony

  3. Le Bonheur Neuroscience Institute, LeBonheur Children’s Hospital, Memphis, TN, USA

    Nir Shimony

  4. Department of Neurological Surgery, University of Tennessee Health Science Center, Memphis, TN, USA

    Nir Shimony & Frederick A. Boop

  5. Department of Neurosurgery, Johns Hopkins University, Baltimore, MD, USA

    Nir Shimony

  6. Semmes Murphey Clinic, Memphis, TN, USA

    Nir Shimony

  7. Global Program, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Frederick A. Boop

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  3. Frederick A. Boop
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All authors wrote the main manuscript text, prepared references, and reviewed the manuscript.

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Correspondence to Frederick A. Boop.

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Boop, S.H., Shimony, N. & Boop, F.A. Review and update on pediatric ependymoma. Childs Nerv Syst 39, 2667–2673 (2023). https://doi.org/10.1007/s00381-023-06091-z

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