If the medical evidence uses a classification other than the WHO classification system
or a tumor is not listed in the 2007 version of the WHO classification system, refer
to the tables below from the 2016 version of the WHO classification system to help
determine the tumor grade. If the tumor is WHO grade I, evaluate the tumor under the
criteria in listings 11.05 and 111.05. (See DI 34001.030 and DI
34005.111.) If the tumor is WHO grade II, evaluate the tumor under listing 13.13A3 or 113.13C.
If the tumor is grade III or IV under the WHO or another classification system, consider
the tumor to be highly malignant and evaluate it under the criteria in listings 13.13A1
or 13.13A2 and 113.13A or 113.13B. As in the 2007 version, the 2016 version of the
WHO classification system is organized according to the cell type or anatomical structure
of the central nervous system where the tumor originated.[9]
Table E. WHO Grade I Tumors
Type of tumor
|
Name of tumor
|
Grade of tumor
|
Other Astrocytic tumors
|
Pilocytic astrocytoma[10]
|
I
|
|
Subependymal giant cell astrocytoma
|
I
|
Ependymal tumors
|
Myxopapillary ependymoma
|
I
|
|
Subependymoma
|
I
|
Other Gliomas
|
Angiocentric glioma
|
I
|
Choroid Plexus tumors
|
Choroid plexus papilloma
|
I
|
Neuronal and Mixed Neuronal-glial tumors
|
Desmoplastic infantile astrocytoma and ganglioglioma
|
I
|
|
Dysembryoplastic neuroepithelial tumor (DNET)
|
I
|
|
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Ducios)
|
I
|
|
Gangliocytoma
|
I
|
|
Papillary glioneuronal tumor
|
I
|
|
Paraganglioma of the filum terminale
|
I
|
|
Rosette-forming glioneuronal tumor of the fourth ventricle
|
I
|
Tumors of the Pineal Region
|
Pineocytoma
|
I
|
Tumors of Cranial and Paraspinal Nerves
|
Cellular schwannoma
|
I
|
|
Melanotic schwannoma
|
I
|
|
Plexiform schwannoma
|
I
|
|
Plexiform neurofibroma
|
I
|
|
Atypical neurofibroma
|
I
|
|
Perineurioma
|
I
|
|
Epithelioid peripheral nerve sheath tumor
|
I
|
Tumors of Meningiomas Cells
|
Meningioma
|
I
|
|
Meningothelial meningioma
|
I
|
|
Fibrous meningioma
|
I
|
|
Microcystic meningioma
|
I
|
|
Angiomatous meningioma
|
I
|
|
Psammomatous meningioma
|
I
|
|
Secretory meningioma
|
I
|
|
Lymphoplasmacyte-rich meningioma
|
I
|
|
Metaplastic meningioma
|
I
|
Mesenchymal, Non-Meningothelial tumors
|
Angiolipoma
|
I
|
|
Chondroma
|
I
|
|
Desmoid-type fibromatosis
|
I
|
|
Haemangioblastoma
|
I
|
|
Hibernoma
|
I
|
|
Leiomyoma
|
I
|
|
Lipoma
|
I
|
|
Osteochondroma
|
I
|
|
Osteoma
|
I
|
|
Rhabdomyoma
|
I
|
|
Solitary fibrous tumor of the dura/haemangiopericytoma[11]
|
I
|
Melanocytic lesions
|
Meningeal melanocytosis
|
I
|
|
Meningeal melanocytoma
|
I
|
Teratomas
|
Mature teratoma
|
I
|
Tumors of the Sellar Region
|
Adamantinomatous craniopharyngioma
|
I
|
|
Adamantinomatous craniopharyngioma
|
I
|
|
Papillary craniopharyngioma
|
I
|
|
Granular cell tumor
|
I
|
|
Papillary craniopharyngioma
|
I
|
|
Pituicytoma
|
I
|
|
Spindle cell oncocytoma
|
I
|
Table F. WHO Grade II Tumors
Type of tumor
|
Name of tumor
|
Grade of tumor
|
Diffuse Astrocytic and Oligodendroglial tumors
|
Diffuse astrocytoma, IDH-mutant
|
II
|
|
Diffuse astrocytoma, IDH-mutant, gemistocytic
|
II
|
|
Diffuse astrocytoma, IDH-wildtype
|
II
|
|
Diffuse astrocytoma, not otherwise specified (NOS)
|
II
|
|
Oligoastrocytoma, not otherwise specified (NOS)
|
II
|
|
Oligodendroglioma, IDH-mutant, 1p19q co-deleted
|
II
|
|
Oligodendroglioma, not otherwise specified (NOS)
|
II
|
Other Astrocytic tumors
|
Pilomyxoid astrocytoma
|
II
|
|
Pleomorphic xanthoastrocytoma
|
II
|
Ependymal tumors
|
Papillary
|
II
|
|
Clear cell
|
II
|
|
Tanycytic
|
II
|
|
RELA fusion-positive
|
II
|
Other gliomas
|
Chordoid glioma of the third ventricle
|
II
|
Choroid Plexus tumors
|
Atypical choroid plexus papilloma
|
II
|
Neuronal and Mixed Neuronal-glial tumors
|
Central neurocytoma
|
II
|
|
Cerebellar liponeurocytoma
|
II
|
|
Extraventricular neurocytoma
|
II
|
Tumors of the Pineal Region
|
Pineal parenchymal tumor of intermediate differentiation[12]
|
II
|
|
Papillary tumor[13]
|
II
|
Tumors of the Cranial and Paraspinal Nerves
|
Epithelioid malignant peripheral nerve sheath tumor (MPNST)[14]
|
II
|
|
MPNST with perineural differentiation
|
II
|
Tumors of Meningothelial cells
|
Atypical meningioma
|
II
|
|
Chordoid meningioma
|
II
|
|
Clear cell meningioma
|
II
|
Mesenchymal, Non-Meningothelial tumors
|
Solitary fibrous tumor of the dura/haemangiopericytoma
|
II
|
Table G. WHO Grade III Tumors
Type of tumor
|
Name of tumor
|
Grade of tumor
|
Diffuse Astrocytic and Oligodendroglial tumors
|
Anaplastic astrocytoma, IDH-mutant[15]
|
III
|
|
Anaplastic astrocytoma, not otherwise specified (NOS)
|
III
|
|
Anaplastic oligoastrocytoma, not otherwise specified (NOS)
|
III
|
|
Anaplastic oligodendroglioma, IDH mutant, 1p19q co-deleted
|
III
|
|
Anaplastic oligodendroglioma, not otherwise specified (NOS)
|
III
|
Other Astrocytic tumors
|
Anaplastic pleomorphic xanthoastrocytoma
|
III
|
Ependymal tumors
|
Anaplastic ependymoma
|
III
|
Choroid Plexus tumors
|
Choroid plexus carcinoma
|
III
|
Neuronal and Mixed Neuronal-Glial tumors
|
Anaplastic ganglioglioma
|
III
|
Tumors of the Pineal Region
|
Pineal parenchymal tumor of intermediate differentiation
|
III
|
|
Papillary tumor of the pineal region
|
III
|
Tumors of the Cranial and Paraspinal Nerves
|
Epithelioid malignant nerve sheath tumor (MPNST)
|
III
|
|
MPNST with perineural differentiation
|
III
|
Tumors of Meningothelial Cells
|
Atypical meningioma
|
III
|
|
Papillary meningioma
|
III
|
|
Rhabdoid meningioma
|
III
|
Mesenchymal, Non-Meningothelial tumors
|
Epithelioid haemangioendothelioma
|
III
|
|
Angiosarcoma
|
III
|
|
Liposarcoma (intracranial)
|
III
|
|
Ewing sarcoma/ETMR
|
III
|
|
Fibrosarcoma
|
III
|
|
Kaposi sarcoma
|
III
|
|
Leiomyosarcoma
|
III
|
|
Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma
|
III
|
|
Rhabdomyosarcoma
|
III
|
|
Solitary fibrous tumor of the dura/haemangiopericytoma
|
III
|
Table H. WHO Grade IV Tumors
Type of tumor
|
Name of tumor
|
Grade of tumor
|
Glioblastoma
|
Glioblastoma, IDH wildtype
|
IV
|
|
Giant cell glioblastoma, IDH wildtype
|
IV
|
|
Gliosarcoma, IDH wildtype
|
IV
|
|
Glioblastoma, IDH mutant
|
IV
|
|
Glioblastoma, not otherwise specified (NOS)
|
IV
|
|
Diffuse midline glioma, H3K27M-mutant
|
IV
|
Tumors of the Pineal Region
|
Pineoblastoma
|
IV
|
Embryonal tumors
|
Medulloblastoma, WNT-activated
|
IV
|
|
Medulloblastoma, SHH-activated & TP53-mutant
|
IV
|
|
Medulloblastoma, SHH-activated & TP53 wildtype
|
IV
|
|
Medulloblastoma, classic
|
IV
|
|
Medulloblastoma, desmoplastic/nodular
|
IV
|
|
Medulloblastoma, extensive nodularity
|
IV
|
|
Medulloblastoma, large cell/anaplastic
|
IV
|
|
Medulloblastoma, not otherwise specified (NOS)
|
IV
|
|
CNS neuroblastoma
|
IV
|
|
CNS ganglioneuroblastoma
|
IV
|
|
Embryonal tumor with multilayer rosettes (ETMR), C19MC-altered
|
IV
|
|
Embryonal tumor with multilayer rosettes (ETMR), not otherwise specified (NOS)
|
IV
|
|
Medulloepithelioma
|
IV
|
|
Atypical teratoid/rhabdoid tumor
|
IV
|
|
CNS embryonal tumor, not otherwise specified (NOS)
|
IV
|
Tumors of Cranial and Paraspinal Nerves
|
Epithelioid malignant nerve sheath tumor
|
IV
|
|
MPNST with perineural differentiation
|
IV
|