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Updated: Jan 10, 2026

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Ependymal Tumors.

Adam G Thomas1, Arpita Chattopadhyay1, Rob A Dineen2

  • 1Department of Neuroradiology, Nottingham University Hospitals NHS Trust, Nottingham, UK.

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|November 22, 2025
PubMed
Summary
This summary is machine-generated.

The 2021 WHO classification refined ependymoma grouping by location and molecular features. New subtypes like ZFTA-fusion and MYCN-altered tumors have distinct imaging and prognostic implications for brain and spinal tumors.

Keywords:
Myxopapillary ependymomaPosterior fossa ependymomaSpinal ependymomaSpinal ependymoma MYCN-AlteredSubependymomaSupratentorial ependymomaYAP1-FusionZFTA-Fusion

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Area of Science:

  • Neuro-oncology
  • Molecular Pathology
  • Radiology

Background:

  • The World Health Organization (WHO) classification of CNS tumors is crucial for diagnosis and treatment.
  • Ependymal tumors represent a significant group of pediatric and adult brain tumors.
  • Previous classifications lacked detailed molecular and anatomical stratification.

Purpose of the Study:

  • To summarize the key changes in the 2021 WHO classification of ependymal tumors.
  • To highlight the new molecular subgroups and their associated imaging features.
  • To outline the prognostic implications of these updated classifications.

Main Methods:

  • Review of the 2021 WHO classification guidelines for CNS tumors.
  • Analysis of molecular subgroups (e.g., ZFTA-fusion, YAP1-fusion, methylation profiles, MYCN-altered).
  • Correlation of molecular and anatomical findings with characteristic imaging features (e.g., cystic, hemorrhagic, enhancement patterns, edema, diffusion-restriction).

Main Results:

  • Ependymal tumors are now categorized into supratentorial, posterior fossa, and spinal locations.
  • Supratentorial ependymomas include ZFTA-fusion (cystic, hemorrhagic, edema) and YAP1-fusion (peripheral enhancement, diffusion-restriction) types.
  • Posterior fossa ependymomas are stratified by methylation into groups A and B with differing prognoses.
  • Spinal ependymomas now include MYCN-altered tumors with poor prognosis and advanced presentation.

Conclusions:

  • The 2021 WHO classification provides a more precise framework for ependymoma diagnosis and management.
  • Molecular subgroups significantly influence the imaging appearance and clinical outcomes of ependymomas.
  • Accurate classification based on location and molecular alterations is essential for targeted therapy and prognostic assessment.