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Review
. 2024 Mar 13:17:1268038.
doi: 10.3389/fnmol.2024.1268038. eCollection 2024.

Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?

Antonio d'Amati  1   2   3   4 , Lavinia Bargiacchi  3 , Sabrina Rossi  5 , Andrea Carai  6 , Luca Bertero  7 , Valeria Barresi  8 , Maria Elena Errico  9 , Anna Maria Buccoliero  10 , Sofia Asioli  11 , Gianluca Marucci  12 , Giada Del Baldo  13 , Angela Mastronuzzi  13 , Evelina Miele  13 , Federica D'Antonio  13 , Elisabetta Schiavello  14 , Veronica Biassoni  14 , Maura Massimino  14 , Marco Gessi  4 , Manila Antonelli  3   14 , Francesca Gianno  3   14
Affiliations
Review

Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?

Antonio d'Amati et al. Front Mol Neurosci. .

Erratum in

Abstract

The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, established new approaches to both CNS tumor nomenclature and grading, emphasizing the importance of integrated diagnoses and layered reports. This edition increased the role of molecular diagnostics in CNS tumor classification while still relying on other established approaches such as histology and immunohistochemistry. Moreover, it introduced new tumor types and subtypes based on novel diagnostic technologies such as DNA methylome profiling. Over the past decade, molecular techniques identified numerous key genetic alterations in CSN tumors, with important implications regarding the understanding of pathogenesis but also for prognosis and the development and application of effective molecularly targeted therapies. This review summarizes the major changes in the 2021 fifth edition classification of pediatric CNS tumors, highlighting for each entity the molecular alterations and other information that are relevant for diagnostic, prognostic, or therapeutic purposes and that patients' and oncologists' need from a pathology report.

Keywords: WHO classification; brain tumors; molecular biology; neuro-oncology; neuropathology; pediatric CNS tumors.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

Figure 1
Figure 1
Map of pediatric CNS tumors, according to age of onset (left column) and location (first row). CNS WHO grade and relevant molecular features of each entity are presented in the figure, belonging to the groups of diffuse low-grade gliomas, diffuse high-grade gliomas, ependymomas, and embryonal tumors.
See this image and copyright information in PMC

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Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This study was supported by “Associazione Italiana per la lotta al Neuroblastoma” and “L’abbraccio di EDO” Foundations.