Table 7.

Newly Recognized Tumor Types in the 2021 WHO Classification of Tumors of the Central Nervous System

Newly Recognized Tumor Types
Diffuse astrocytoma, MYB- or MYBL1-altered
Polymorphous low-grade neuroepithelial tumor of the young
Diffuse low-grade glioma, MAPK pathway-altered
Diffuse hemispheric glioma, H3 G34-mutant
Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
Infant-type hemispheric glioma
High-grade astrocytoma with piloid features
Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (provisional type)
Myxoid glioneuronal tumor
Multinodular and vacuolating neuronal tumor
Supratentorial ependymoma, YAP1 fusion-positive
Posterior fossa ependymoma, group PFA
Posterior fossa ependymoma, group PFB
Spinal ependymoma, MYCN-amplified
Cribriform neuroepithelial tumor (provisional type)
CNS neuroblastoma, FOXR2-activated
CNS tumor with BCOR internal tandem duplication
Desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant
Intracranial mesenchymal tumor, FET-CREB fusion positive (provisional type)
CIC-rearranged sarcoma
Primary intracranial sarcoma, DICER1-mutant
Pituitary blastoma