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 1).
Differential diagnosis
pediatric-type diffuse low-grade gliomas:
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 gliomas 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 2).