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Mesenchymal, Non-Meningothelial Tumors
Aligned with soft tissue/bone classification; includes vascular, fibroblastic, myogenic, and chondro-osseous entities.
Subtypes3
Grade Range
G1G2G3
Subtypes
Solitary fibrous tumor (SFT)
WHO Grade 1WHO Grade 2WHO Grade 3
Formerly hemangiopericytoma. Dural-based, mimicking meningioma.
Diagnostic Molecular Markers
NAB2::STAT6 gene fusion
Locations:
Hemangioblastoma
WHO Grade 1
Highly vascular tumor, classically in the cerebellum.
Diagnostic Molecular Markers
VHL mutation
Locations:
Chordoma
WHO Grade 2WHO Grade 3
Arises from notochord remnants, classically at the clivus / skull base.
Diagnostic Molecular Markers
Brachyury (TBXT) expression
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.