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The cranial and spinal meninges are complex protective structures surrounding the central nervous system (CNS), consisting of the brain and spinal cord. These meninges consist of the dura mater, the arachnoid mater, and the pia mater. They protect the CNS, provide structural support, and aid in circulating cerebrospinal fluid (CSF).
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Neurulation is the embryological process which forms the precursors of the central nervous system and occurs after gastrulation has established the three primary cell layers of the embryo: ectoderm, mesoderm, and endoderm. In humans, the majority of this system is formed via primary neurulation, in which the central portion of the ectoderm—originally appearing as a flat sheet of cells—folds upwards and inwards, sealing off to form a hollow neural tube. As development proceeds, the...
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Related Experiment Video

Updated: Aug 16, 2025

Author Spotlight: A Single-Entry Point Endoscopic Intraventricular Approach for Third Ventriculostomy and Pineal Biopsy
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Ependymomas.

Luca Bertero1, Alessia Andrea Ricci1, Cristian Tampieri1

  • 1Pathology Unit, Department of Medical Sciences, University of Turin, Turin, Italy.

Pathologica
|December 19, 2022
PubMed
Summary
This summary is machine-generated.

The WHO CNS tumor classification now integrates new molecular data for ependymomas. Key updates include renaming RELA fusion-positive to ZFTA fusion-positive and adding YAP1 fusion-positive supratentorial ependymomas.

Keywords:
WHO classificationependymomamolecular pathologymyxopapillary ependymomasubependymoma

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Area of Science:

  • Neuro-oncology
  • Molecular Pathology
  • Cancer Classification

Background:

  • Ependymal neoplasms originate from cells lining the brain's ventricles and spinal canal.
  • Recent advancements in understanding oncogenesis and molecular features have necessitated classification updates.
  • The 5th edition of the WHO Classification of CNS Tumours incorporates significant new data.

Purpose of the Study:

  • To present the recent changes in the classification of ependymal tumors.
  • To summarize the current diagnostic framework for ependymal neoplasms based on the latest WHO updates.

Main Methods:

  • Review of novel data on ependymoma oncogenesis, molecular characteristics, and clinical correlations.
  • Analysis of the 5th edition WHO Classification of Central Nervous System Tumours.
  • Integration of molecular profiling and immunohistochemical surrogates for classification.

Main Results:

  • Supratentorial ependymomas: RELA fusion-positive renamed ZFTA fusion-positive; YAP1 fusion-positive subtype added.
  • Posterior fossa ependymomas: Subtyped into Type A or B using molecular profiling or H3 K27me3 surrogate.
  • Spinal ependymomas: New subtype with MYCN amplification and poor prognosis identified; Myxopapillary ependymoma reclassified as Grade 2.

Conclusions:

  • The 5th edition WHO classification provides a refined framework for diagnosing ependymal tumors.
  • Molecular insights are crucial for accurate subtyping and prognostication of ependymal neoplasms.
  • Updated classification reflects a better understanding of ependymoma heterogeneity and clinical behavior.