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Major Features of the 2021 WHO Classification of CNS Tumors
Heather L Smith1, Nitin Wadhwani2, Craig Horbinski3,4
1Department of Pathology, University of Chicago, Chicago, IL, USA.
Abstract:
Advances in the understanding of the molecular biology of central nervous system (CNS) tumors prompted a new World Health Organization (WHO) classification scheme in 2021, only 5 years after the prior iteration. The 2016 version was the first to include specific molecular alterations in the diagnoses of a few tumors, but the 2021 system greatly expanded this approach, with over 40 tumor types and subtypes now being defined by their key molecular features. Many tumors have also been reconceptualized into new "supercategories," including adult-type diffuse gliomas, pediatric-type diffuse low- and high-grade gliomas, and circumscribed astrocytic gliomas. Some entirely new tumors are in this scheme, particularly pediatric tumors. Naturally, these changes will impact how CNS tumor patients are diagnosed and treated, including clinical trial enrollment. This review addresses the most clinically relevant changes in the 2021 WHO book, including diffuse and circumscribed gliomas, ependymomas, embryonal tumors, and meningiomas.
Insights
The 2021 World Health Organization (WHO) classification for central nervous system (CNS) tumors integrates molecular features, defining over 40 tumor types and subtypes. These advancements impact CNS tumor diagnosis, treatment, and clinical trial enrollment.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Genomics
Background:
- The 2021 World Health Organization (WHO) classification for central nervous system (CNS) tumors represents a significant shift from previous iterations.
- Molecular alterations were minimally included in the 2016 classification, but are now central to defining CNS tumors in the 2021 scheme.
Purpose of the Study:
- To review the most clinically relevant changes introduced in the 2021 WHO classification of CNS tumors.
- To highlight the impact of molecularly defined tumor types on diagnosis and treatment strategies.
Main Methods:
- Review of the 2021 WHO classification of CNS tumors.
- Analysis of key molecular alterations defining new tumor types and supercategories.
- Discussion of implications for clinical practice, including diagnosis and trial enrollment.
Main Results:
- The 2021 WHO classification extensively incorporates molecular features, defining over 40 tumor types and subtypes.
- New supercategories like adult-type diffuse gliomas and pediatric-type diffuse gliomas have been established.
- Several new tumor entities, particularly in pediatric CNS tumors, are introduced.
Conclusions:
- The 2021 WHO classification signifies a paradigm shift towards molecularly defined CNS tumors.
- These changes necessitate updated diagnostic and therapeutic approaches for CNS tumor patients.
- Understanding these molecular classifications is crucial for future clinical trial design and patient management.
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