====== Diffuse astrocytoma MYB or MYBL1 altered ====== [[Diffuse astrocytoma]] [[MYB]] or [[MYBL1]] altered is a [[pediatric brain tumor]] type included in the [[World Health Organization Classification of Tumors of the Central Nervous System 2021]] ((Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-1251. doi: 10.1093/neuonc/noab106. PMID: 34185076; PMCID: PMC8328013.)). ===== Differential diagnosis ===== pediatric-type diffuse low-grade gliomas: [[angiocentric glioma]] [[polymorphous low-grade neuroepithelial tumor of the young]] [[diffuse low-grade glioma MAPK pathway-altered]] pediatric-type diffuse high-grade gliomas: diffuse midline glioma, H3 K27-altered diffuse hemispheric glioma, H3 G34-mutant diffuse pediatric-type high-grade glioma, H3-wild type, and IDH-wild type infant-type hemispheric glioma [[dysembryoplastic neuroepithelial tumor]] (DNET) Specific [[DNT]] is a homogeneous group of tumors sharing characteristics of [[pediatric low-grade glioma]]s a quiet genome with a recurrent genomic alteration in the [[RAS]]-[[MAPK]] [[signaling pathway]], a distinct [[DNA methylation]] profile, a good prognosis but showing progression in some cases. The "non-specific/diffuse DNTs" subgroup encompasses various recently described histo-molecular entities, such as [[polymorphous low-grade neuroepithelial tumor of the young]] and [[Diffuse astrocytoma MYB or MYBL1 altered]] ((Pagès M, Debily MA, Fina F, Jones DTW, Saffroy R, Castel D, Blauwblomme T, Métais A, Bourgeois M, Lechapt-Zalcman E, Tauziède-Espariat A, Andreiuolo F, Chrétien F, Grill J, Boddaert N, Figarella-Branger D, Beroukhim R, Varlet P. The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases. Neuropathol Appl Neurobiol. 2022 Jul 14:e12834. doi: 10.1111/nan.12834. Epub ahead of print. PMID: 35836307.)).