The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low‐grade gliomas and glioneuronal tumors. (25th February 2022)

• Record Type: Journal Article
• Authors: Bale, Tejus A.; Rosenblum, Marc K.
• Is Part Of: Brain pathology. Volume 32:Number 4(2022)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2022-02-25
• Subjects: 2021 CNS WHO -- glioneuronal tumors -- low‐grade gliomas; Nervous system -- Diseases -- Periodicals; Brain -- Diseases -- Periodicals; Neurology -- Periodicals; Brain Diseases -- Periodicals; Cerveau -- Maladies -- Périodiques; Système nerveux -- Maladies -- Périodiques; Neurologie -- Périodiques; 616.805
• DOI: 10.1111/bpa.13060
• Languages: English
• ISSNs: 1015-6305

Abstract

Abstract: The 2021 5th edition of the WHO Classification of Tumors of the Central Nervous System reflects the discovery of genetic alterations underlying many central nervous system (CNS) neoplasms. Insights gained from technologic advances and novel applications in molecular diagnostics, including next‐generation sequencing and DNA methylation‐based profiling, coupled with the recognition of clinicopathologic correlates, have prompted substantial changes to CNS tumor classification; this is particularly true for pediatric low‐grade gliomas and glioneuronal tumors (pLGG/GNTs). The 2021 WHO now classifies gliomas, glioneuronal tumors and neuronal tumors into 6 families, three of which encompass pLGG/LGNTs: "Pediatric type diffuse low‐grade gliomas, " "circumscribed astrocytic gliomas, " and "glioneuronal and neuronal tumors." Among these are six newly recognized tumor types: "diffuse astrocytoma, MYB or MYBL1 ‐altered"; "polymorphous low grade neuroepithelial tumor of the young (PLNTY)"; "diffuse low‐grade glioma‐MAPK altered"; "Diffuse glioneuronal tumor with oligodendroglioma‐like features and nuclear clusters (DGONC)"; "myxoid glioneuronal tumor (MGT)"; and "multinodular and vacuolating neuronal tumor (MVNT)." We review these newly recognized entities in the context of general changes to the WHO schema, discuss implications of the new classification for treatment of pLGG/LGNT, and consider strategies for molecular testing and interpretation. Abstract : The 5th edition of the WHO Classification of Tumors of the Central Nervous System now classifies gliomas, glioneuronal tumors and neuronal tumors into 6 families, among which are six newly pediatric low‐grade gliomas and glioneuronal tumors: "diffuse astrocytoma, MYB or MYBL1‐altered"; "polymorphous low grade neuroepithelial tumor of the young (PLNTY)"; "diffuse low‐grade glioma‐ MAPK altered"; "Diffuse glioneuronal tumor with oligodendroglioma‐like features and nuclear clusters (DGONC)"; "myxoid glioneuronal tumor (MGT)"; and "multinodular and vacuolating neuronal tumor (MVNT)." We review these newly recognized entities in the context of general changes to the WHO schema, discuss implications of the new classification for treatment of pLGG/LGNT, and consider strategies for molecular testing and interpretation.