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Cranial and Paraspinal Nerve Tumors
Nerve sheath neoplasms and related tumors, including paragangliomas.
Subtypes3
Grade Range
G1G2G3G4
Subtypes
Schwannoma
WHO Grade 1
Benign nerve sheath tumor. Most common is vestibular schwannoma (CN VIII) at the cerebellopontine angle.
Diagnostic Molecular Markers
NF2 inactivation (Chr 22q loss)
Locations:
Neurofibroma
WHO Grade 1
Associated with Neurofibromatosis Type 1. Affects cranial, spinal, and peripheral nerve roots.
Diagnostic Molecular Markers
NF1 inactivation
Locations:
Malignant peripheral nerve sheath tumor (MPNST)
WHO Grade 2WHO Grade 3WHO Grade 4
Aggressive malignancy of major nerve trunks and plexuses.
Diagnostic Molecular Markers
NF1 lossCDKN2A/B lossPRC2 inactivation
Locations:
Primary Locations
Clinical Note
Grading and diagnostic criteria listed here are based strictly on the 2021 WHO Classification of Tumours of the Central Nervous System (5th Edition). Many entities now require integrated phenotypic and genotypic characterization for a final diagnosis.