Table 1.
Glioma types of clinical interest in children and adolescents, as per the 2021 WHO Classification of Tumors of the Central Nervous System, Fifth Edition.
| Gliomas of clinical interest in children and adolescents | New entity (2021 CNS WHO) | Genetic/molecular alterations |
|---|---|---|
| Pediatric-type diffuse low-grade gliomas | ||
| Diffuse astrocytoma, MYB- or MYBL1-altered | x | MYB, MYBL1 |
| Angiocentric glioma | MYB | |
| Polymorphous low-grade neuroepithelial tumor of the young | x | BRAF, FGFR family |
| Diffuse low-grade glioma, MAPK pathway-altered | x | FGFR1, BRAF |
| Pediatric-type diffuse high-grade gliomas | ||
| Diffuse midline glioma, H3 K27-altered | refined | H3 K27, TP53, ACVR1, PDGFRA, EGFR, EZHIP |
| Diffuse hemispheric glioma, H3 G34-mutant | x | H3 G34, TP53, ATRX |
| Diffuse pediatric-type high-grade glioma, H3-wild-type, and IDH-wild-type | x | IDH-wild-type, H3-wild-type, PDGFRA, MYCN, EGFR |
| Infant-type hemispheric glioma | x | NTRK, ALK, ROS, MET |
| Circumscribed astrocytic gliomas | ||
| Pilocytic astrocytoma | KIAA1549-BRAF, BRAF, NF1 | |
| High-grade astrocytoma with piloid features | x | BRAF, NF1, ATRX, CDKN2A/B |
| Pleomorphic xanthoastrocytoma | BRAF, CDKN2A/B | |
| Subependymal giant cell astrocytoma | TSC1, TSC2 | |
| Astroblastoma, MN1-altered | MN1 | |
Newly defined entities are marked in the second column. Typical genetic alterations are listed in the third column for each tumor type. Adapted from (3, 4). NB: tumors that are exclusively found in adults, although present in the 2021 WHO CNS classification, are not listed in this table. Glioneuronal tumors and ependymomas, although of pediatric interest, are not listed.