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2021 WHO classification of gliomas, glioneuronal tumors, and neuronal tumors

2021 WHO classification of gliomas, glioneuronal tumors, and neuronal tumors
Adult-type diffuse gliomas
  • Astrocytoma, IDH-mutant
  • Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Glioblastoma, IDH-wildtype
Pediatric-type diffuse low-grade gliomas
  • Diffuse astrocytoma, MYB- or MYBL1-altered
  • 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-wildtype and IDH-wildtype
  • Infant-type hemispheric glioma
Circumscribed astrocytic glioma
  • Pilocytic astrocytoma
  • High-grade astrocytoma with piloid features
  • Pleomorphic xanthoastrocytoma
  • Subependymal giant cell astrocytoma
  • Chordoid glioma
  • Astroblastoma, MN1-altered
Glioneuronal and neuronal tumors
  • Ganglioglioma
  • Gangliocytoma
  • Desmoplastic infantile ganglioglioma/desmoplastic infantile astrocytoma
  • Dysembryoplastic neuroepithelial tumor
  • Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters
  • Papillary glioneuronal tumor
  • Rosette-forming glioneuronal tumor
  • Myxoid glioneuronal tumor
  • Diffuse leptomeningeal glioneuronal tumor
  • Multinodular and vacuolating neuronal tumor
  • Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
  • Central neurocytoma
  • Extraventricular neurocytoma
  • Cerebellar liponeurocytoma
Ependymal tumors
  • Supratentorial ependymoma
  • Supratentorial ependymoma, ZFTA fusion-positive
  • Supratentorial ependymoma, YAP1 fusion-positive
  • Posterior fossa ependymoma
  • Posterior fossa group A (PFA) ependymoma
  • Posterior fossa group B (PFB) ependymoma
  • Spinal ependymoma
  • Spinal ependymoma, MYCN-amplified
  • Myxopapillary ependymoma
  • Subependymoma
Gliomas, glioneuronal tumors, and neuronal tumors in the WHO Classification of Central Nervous System Tumours, 5th edition. Shaded categories contain pediatric low-grade gliomas.
H3: histone 3; IDH: isocitrate dehydrogenase; MAPK: mitogen-activated protein kinase; WHO: World Health Organization.
Adapted from Central Nervous System Tumours: WHO Classification of Tumours, 5th ed, Vol. 6, WHO Classification of Tumours Editorial Board. Copyright © 2021 International Agency for Research on Cancer. https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Central-Nervous-System-Tumours-2021 (Accessed on October 31, 2024).
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