4.5 Review

The 2021 WHO classification of central nervous system tumors: what neurologists need to know

Journal

CURRENT OPINION IN NEUROLOGY
Volume 35, Issue 6, Pages 764-771

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WCO.0000000000001109

Keywords

brain tumor; central nervous system neoplasm; molecular diagnostics; neurology; WHO classification

Funding

  1. National Institutes of Health [R01 CA214928, R01 CA247905, P50 CA221747, U01 CA217613, U01 CA199288]

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The recently published WHO Classification of Tumours, Central Nervous System Tumours, Fifth Edition introduces significant clinical changes based on improved understanding of the molecular basis of brain tumor types. These changes provide neurologists with opportunities for individualized treatment options and the discovery of novel therapeutics.
Purpose of reviewThe recently published WHO Classification of Tumours, Central Nervous System Tumours, Fifth Edition (WHO CNS-5) introduces substantial clinically relevant changes based on improved understanding of the molecular underpinnings of brain tumor types as biological entities. This review highlights pertinent changes for practicing neurologists.Recent findingsDiffuse gliomas are now divided into adult and pediatric types. Adult types are greatly simplified, being classified into three groups based on IDH and 1p/19q status, with molecular grading criteria now included. Pediatric types are divided into low-grade or high-grade and further classified based on molecular features corresponding to clinical behavior. While still recognizing previous morphological subtypes, meningioma is now a single tumor type, with greatly advanced correlations between molecular alterations, locations, morphologic subtypes, and grades. For the first time, ependymomas are classified based on integration of anatomical location, histopathology, and molecular alterations. Importantly, WHO CNS-5 includes a number of new tumor types that have similar clinicopathologic features and are grouped together by their distinctive molecular characteristics.The classification of CNS tumors according to objective, reproducible molecular genetic alterations, provides greater opportunity for neurologists to offer individualized treatment options, enroll homogenous patient populations into clinical trials, and ultimately discover novel therapeutics.

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