Diffuse astrocytoma MYB or MYBL1 altered [Neurosurgery Wiki]

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====== 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.)).