- Diffuse gliomas, separated from the common group of glial tumours, as diffuse astrocytic and oligodendroglial tumours
- Restructured with incorporation of genetically defined entities
- Medulloblastomas are restructured with incorporation of genetically defined entities
- Other embryonal tumors are also restructured, with incorporation of genetically defined entities
- The term “primitive neuroectodermal tumor” is removed
- Ependymoma - genetically defined variant of RELA fusion positive incorporated.
- Newly recognized entities, variants and patterns added:
- IDH-wildtype and IDH-mutant glioblastoma (entities)
- Diffuse midline glioma, H3 K27M–mutant (entity)
- Embryonal tumour with multilayered rosettes, C19MC-altered (entity)
- Ependymoma, RELA fusion–positive (entity)
- Diffuse leptomeningeal glioneuronal tumor (entity)
- Anaplastic PXA (entity)
- Epithelioid glioblastoma (variant)
- Glioblastoma with primitive neuronal component (pattern)
- Multinodular and vacuolated pattern of ganglion cell tumor (pattern)
- Entities, variants and terms deleted:
- Gliomatosis cerebri
- Protoplasmic and fibrillary astrocytoma variants
- Cellular ependymoma variant
- “Primitive neuroectodermal tumour” terminology
- Addition of brain invasion as a criterion for the diagnosis of atypical meningioma
- Restructuring of solitary fibrous tumor and hemangiopericytoma (SFT/HPC) as one entity with grades from I to III
- Addition of hybrid nerve sheath tumors and separation of melanotic schwannoma from other schwannomas
- Expansion of entities included in hematopoietic/lymphoid tumors of the CNS (lymphomas and histiocytic tumors)
Louis DN, et al. The 2016 World Health Organization Classification of Tumors of the Central
Nervous System: a summary. Acta Neuropathol, 2016;6: 803-820