The WHO 2021 Classification of Central Nervous System tumours: a practical update on what neurosurgeons need to know-a minireview

Abstract

Background: The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours. This classification system is under continuous revision, and progress in the molecular classification of tumours in the central nervous system (CNS) enforced an update of the WHO 2016 classification, and the fifth edition, WHO CNS5, was published in 2021. The aim of this minireview is to highlight important changes in this new edition relevant for the practicing neurosurgeon.

Methods: The sixth volume of the fifth edition of the WHO Blue Books of CNS tumours and related papers formed the basis for this minireview.

Results: Major changes encompass standardisation of tumour grading and nomenclature as well as increased incorporation of molecular markers in the classification of CNS tumours.

Conclusion: Advances in molecular genetics have resulted in more accurate diagnosis and prognosis of CNS tumours, and this minireview summarises important changes implemented in the last edition of WHO classification of CNS tumours important for the practicing neurosurgeon.

Keywords: Biomarker; Brain tumours; Diagnosis; Molecular genetics; Pathology.

Fig. 1

Simplified diagnostic algorithm for diffuse gliomas in adults. Astrocytoma, IDH-wildtype without histopathological and molecular features of glioblastoma is rare, and these tumours should undergo further molecular genetic analyses and methylation profiling. IDH-wildtype gliomas should also be considered for analysis of H3 K27 and H3 G34 mutations (figure inspired by [16])