Table 3.

Newly recognized tumor types, 2021 WHO CNS-5

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