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. 2023 Jan 16;12:1082062. doi: 10.3389/fonc.2022.1082062

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Copyright © 2023 Vallero, Bertero, Morana, Sciortino, Bertin, Mussano, Ricci, Peretta and Fagioli

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Graphic representation of how pediatric DIPGs and DMGs have been classified over the last 15 years, based on the current and the past WHO Classifications of CNS tumors (2, 3, 68). In 2007, DIPG/DMG was not recognized as a distinct entity: data from autopsies and rare biopsies showed that DIPG/DMGs were histologically classifiable as glioblastomas (GBM gr. IV), or less frequently, anaplastic astrocytomas (AA gr. III), low-grade gliomas (mainly diffuse astrocytomas, LGG-DA gr. II), or other rarer histotypes (69). In 2016, the majority of cases were classified as DMG, H3K27M-mutant tumors, although some non-K27M-mutant DMG still remained unclassified. The 2021 WHO CNS Classification unified all cases of pediatric DMGs in which a H3K27 alteration was found (loss of H3 K27me3 trimethylation). Legend: GBM, glioblastoma; AA, anaplastic astrocytoma; LGG, low-grade glioma; DA; diffuse astrocytoma; IDHwt, IDH wild-type; NOS, not otherwise specified; DMG, diffuse midline glioma; DIPG, diffuse intrinsic pontine glioma.