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Ependymoma

  • Christine Hill-Kayser
Chapter
Part of the Practical Guides in Radiation Oncology book series (PGRO)

Abstract

  • Ependymoma represents the third most common primary brain tumor in children and accounts for approximately 10% of brain tumors in children and >20% of spinal cord tumors in children [1].

  • Ependymoma diagnosis peaks between 0 and 4 years of age, and ependymomas are slightly more common in boys than in girls. Overall, they affect 0.26/100,000 children aged 0–14 years [1, 2].

  • Ependymoma may be further characterized according to histopathology as well as location within the CNS (supratentorial brain, infratentorial brain, spinal cord).
    • The World Health Organization (WHO) separates ependymomas into three groups based on histopathologic grade, with several subclassifications. Subclassifications have been recently modified with a 2016 update of the WHO CNS 4th edition; modifications include incorporation of a genetically defined ependymoma variant (RELA fusion-positive). (Table 4.1) [3].

      RELA fusion-positive ependymoma is the first molecular ependymoma variant to be formally recognized. Over two-thirds of supratentorial ependymomas contain oncogenic fusions between RELA (an effector of NF-κB signaling) and an uncharacterized gene, C11orf95, involving chromosome 11q13 [4].

    • Additionally, children are affected by these tumors within specific anatomic areas based on age, with mean age 12.2 years for spinal cord ependymomas, 7.8 years for supratentorial tumors, and 5 years for infratentorial tumors [5].

  • Ependymomas can disseminate throughout the central nervous system, although this is relatively rare, with <15% of ependymoma tumors being associated with dissemination detected either on imaging or via lumbar puncture [6, 7]

  • Surgical resection and focal radiotherapy remain the mainstays of treatment for ependymoma, with the role of chemotherapy remaining investigational.
    • Historically, surgical resection has been regarded as the most crucial aspect of treatment, with improvement in prognosis observed for patients with gross total as compared to incomplete resections [8–10].

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Suggested Reading

  1. 1.
    Overview of Pediatric Ependymoma: Vitanza NA and Partap S. Pediatric Ependymoma. J Child Neurology 1(13), 2015Google Scholar
  2. 2.
    Overview of spinal cord ependymoma: Benesch M, Frappaz D, Massimino M. Spinal cord Ependymoma in Children and Adolescents. Childs Nerv Syst 28: 2017–2028, 2012Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Radiation OncologyPerelman School of Medicine, University of PennsylvaniaPhiladelphiaUSA
  2. 2.The Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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