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Ependymomas in children.

L E Kun1, E H Kovnar, R A Sanford

  • 1Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tenn.

Pediatric Neuroscience
|January 1, 1988
PubMed
Summary

Ependymomas are common pediatric central nervous system tumors. Treatment strategies, including surgery, radiation, and chemotherapy, are evolving, especially for high-grade tumors.

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Area of Science:

  • Pediatric neuro-oncology
  • Central Nervous System (CNS) tumors
  • Ependymoma biology and treatment

Background:

  • Ependymomas constitute 10-12% of pediatric CNS tumors, predominantly in children under five.
  • Most commonly arise in the fourth ventricle region, with ongoing debate on histologic categorization.
  • Anaplastic or high-grade lesions represent about 25% of childhood ependymomas.

Purpose of the Study:

  • To review current understanding and controversies in ependymoma management.
  • To evaluate the efficacy of different treatment modalities for various ependymoma subtypes.
  • To highlight evolving treatment sequences involving surgery, chemotherapy, and radiation.

Main Methods:

  • Literature review of historical and recent data on ependymoma treatment outcomes.
  • Analysis of surgical resection, radiation therapy (local vs. craniospinal), and chemotherapy responses.
  • Examination of clinico-histologic correlations with tumor spread and treatment efficacy.

Main Results:

  • Surgical resection is curative for cauda equina ependymomas; surgery alone achieves 75% disease control for intramedullary tumors.
  • Postoperative irradiation improves survival for intracranial ependymomas from earlier eras.
  • High-grade, infratentorial ependymomas show the highest rate of subarachnoid spread (12% incidence).
  • Chemotherapy shows single-agent responses, but adjuvant use has not yet improved survival; neoadjuvant chemotherapy is being studied.

Conclusions:

  • Treatment strategies for pediatric ependymomas vary based on tumor location and grade.
  • Craniospinal irradiation remains a point of debate due to neuraxis seeding risks.
  • Current research focuses on neoadjuvant chemotherapy, particularly for very young children and high-grade tumors.

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