MPC-17 2021 WHO Classification of Tumors of the CNS, 5th ed

Abstract

The grading of gliomas based on histological features has been a subject of debate for several decades. While the traditional grading system has failed to stratify the risk of IDH-mutant astrocytoma, canonical histological and proliferative markers may be applicable to the risk stratification of IDH-wildtype astrocytoma. Numerous studies have examined molecular markers to obtain more clinically relevant information that will improve the risk stratification of gliomas. The CDKN2A/B homozygous deletion for IDH-mutant astrocytoma and the following three criteria for IDH-wildtype astrocytoma: the concurrent gain of whole chromosome 7 and loss of whole chromosome 10, TERT promoter mutations, and EGFR amplification, were identified as independent molecular markers of the worst clinical outcomes. Therefore, the 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System adopted these molecular markers into the revised grading criteria of IDH-mutant and -wildtype astrocytoma respectively, as a grading system within tumor types. For diffuse gliomas in children, molecular alteration-based classification was adopted, dividing low-grade and high-grade subcategories. New tumor types and subtypes were introduced, some based on DNA methylation profiling. To achieve this novel classification in a resource-limited setting, an integrated diagnosis combining clinical, histological, and molecular information became more important.