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2021 WHO classification of tumours of the central nervous system: a review for the neuroradiologist

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Abstract

The fifth edition of the World Health Organization Classification of Tumours of the Central Nervous System (WHO CNS5) published in 2021 builds on the 2016 edition and incorporates output from the Consortium to Inform Molecular and Practical Approaches to CNS Tumour Taxonomy (cIMPACT-NOW). WHO CNS5 introduces fundamental changes to brain tumour classification through the introduction of new tumour families and types, especially in the paediatric population, and a revision of diagnostic criteria for some of the existing neoplasms. Neuroradiologists are central to brain tumour diagnostics, and it is therefore essential that they become familiar with the key updates. This review aims to summarise the most relevant updates for the neuroradiologist and, where available, discuss the known radiophenotypes of various new tumour types to allow for increased accuracy of language and diagnosis. Of particular importance, WHO CNS5 places greater emphasis on organising tumours by molecular type to reflect biology, as well as to allow for better planning of treatment. The principal updates in adult tumours concern the molecular definition of glioblastoma, restructuring of diffuse gliomas, and the introduction of several new tumour types. The updates to the paediatric classification are protean, ranging from the introduction of new types to establishing separate tumour families for paediatric-type gliomas. This review summarises the most significant revisions and captures the rationale and radiological implications for the major updates.

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Acknowledgements

Olivia Carney and Sniya Sudhakar, Department of Radiology, Great Ormond Street Hospital, for many very helpful discussions.

Funding

No funding was received for this review. TJ is grateful for funding from the Brain Tumour Charity, Children with Cancer UK, Great Ormond Street Hospital Children’s Charity, Olivia Hodson Cancer Fund, Cancer Research UK, and the National Institute of Health Research. All research at Great Ormond Street Hospital NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health is made possible by the NIHR Great Ormond Street Hospital Biomedical Research Centre. ST receives funding from the UCL/UCLH NIHR Biomedical Research Centre. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health.

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Authors and Affiliations

  1. Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

    Cillian McNamara, Kshitij Mankad, Felice D’Arco & Ulrike Löbel

  2. Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK

    Cillian McNamara & Stefanie Thust

  3. Department of Neuroradiology, Imperial College Healthcare NHS Trust, London, UK

    Luke Dixon

  4. Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK

    Clara Limback-Stanic

  5. DBC Programme, UCL GOS Institute of Child Health and Department of Histopathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

    Thomas S. Jacques

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  1. Cillian McNamara
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Correspondence to Cillian McNamara.

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Ethics approval

We obtained our institutional review board approval for including cases in this review study and the study was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Consent was waived for this retrospective study in which only fully anonymised studies are used.

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CMN and KM are joint first authors.

Key points

• The fifth edition of the WHO Classification of Tumours of the Central Nervous System places an increased emphasis on molecular data to reach a comprehensive integrated diagnosis.

• Several new tumour types and subtypes have been introduced, especially within the families of paediatric-type diffuse gliomas and embryonal tumours.

• Diffuse gliomas are now separated into paediatric-type and adult-type based on their underlying molecular differences.

• Diffuse paediatric gliomas are now divided into two families: paediatric-type diffuse low-grade gliomas and paediatric-type diffuse high-grade gliomas.

• Glioblastomas are adult-type tumours and are IDH-wildtype.

• Ependymomas are now classified according to a combination of histopathological and molecular features as well as anatomical location.

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McNamara, C., Mankad, K., Thust, S. et al. 2021 WHO classification of tumours of the central nervous system: a review for the neuroradiologist. Neuroradiology 64, 1919–1950 (2022). https://doi.org/10.1007/s00234-022-03008-6

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