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Ependymal Tumors.

Mariarita Santi^1,2, Angela N Viaene^1,2, Cynthia Hawkins^3,4,5

¹Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

|February 16, 2022

Summary

Pediatric ependymomas (EPN) diagnosis is evolving. Molecular and location-based classification improves risk stratification over traditional histopathology for better patient outcomes.

Keywords:

DNA copy number variations ependymoma epigenetic pediatric transcriptional profiles

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Published on: February 22, 2015

Area of Science:

Neuro-oncology
Pediatric neurosurgery
Molecular pathology

Background:

Ependymomas (EPN) are common pediatric brain tumors with challenging histopathologic grading.
Traditional grading shows poor reproducibility and limited correlation with patient outcomes.
EPN exhibit site-specific molecular alterations, necessitating a revised classification.

Purpose of the Study:

To provide a practical diagnostic approach for ependymomas.
To guide selection of molecular and immunohistochemical markers.
To facilitate rapid, accurate diagnosis for improved patient risk stratification and clinical decision-making.

Main Methods:

Review of current literature on ependymoma classification and diagnosis.
Emphasis on molecular surrogates and immunohistochemical stains.
Integration of basic and advanced molecular techniques.

Main Results:

Molecular and location-based classification is superior to histopathology for risk stratification.
Site-specific genetic and epigenetic alterations define EPN subtypes.
WHO CNS Tumor Classification now incorporates molecular and location-based criteria.

Conclusions:

A molecular and location-based approach refines ependymoma diagnosis and prognosis.
Accurate diagnosis guides therapeutic strategies and improves patient management.
This approach enhances the precision of risk stratification in pediatric ependymomas.