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Review ArticleBrain Tumor Imaging

Meningioma: Molecular Updates from the 2021 World Health Organization Classification of CNS Tumors and Imaging Correlates

Neetu Soni, Manish Ora, Girish Bathla, Denes Szekeres, Amit Desai, Jay J. Pillai and Amit Agarwal
American Journal of Neuroradiology February 2025, 46 (2) 240-250; DOI: https://doi.org/10.3174/ajnr.A8368
Neetu Soni
aFrom the Department of Radiology (N.S., J.J.P., A.D., A.A.), Mayo Clinic, Jacksonville, Florida
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Manish Ora
bDepartment of Nuclear Medicine (M.O.), Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, India
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Girish Bathla
cDepartment of Radiology (G.B., J.P.), Mayo Clinic, Rochester, Minnesota
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Denes Szekeres
dUniversity of Rochester School of Medicine and Dentistry (D.S.), Rochester, New York
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Amit Desai
aFrom the Department of Radiology (N.S., J.J.P., A.D., A.A.), Mayo Clinic, Jacksonville, Florida
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Jay J. Pillai
cDepartment of Radiology (G.B., J.P.), Mayo Clinic, Rochester, Minnesota
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Amit Agarwal
aFrom the Department of Radiology (N.S., J.J.P., A.D., A.A.), Mayo Clinic, Jacksonville, Florida
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Abstract

SUMMARY: Meningiomas, the most common primary intracranial neoplasms, account for more than one-third of primary CNS tumors. While traditionally viewed as benign, meningiomas can be associated with considerable morbidity, and specific meningioma subgroups display more aggressive behavior with higher recurrence rates. The risk stratification for recurrence has been primarily associated with the World Health Organization (WHO) histopathologic grade and extent of resection. However, a growing body of literature has highlighted the value of molecular characteristics in assessing recurrence risk. While maintaining the previous classification system, the 5th edition of the 2021 WHO Classification of Central Nervous System tumors (CNS5) book expands upon the molecular information in meningiomas to help guide management. The WHO CNS5 stratifies meningioma into 3 grades (1−3) based on histopathology criteria and molecular profile. The telomerase reverse transcriptase promoter mutations and cyclin-dependent kinase inhibitor 2A/B (CDKN2A/B) deletions now signify a grade 3 meningioma with increased recurrence risk. Tumor location also correlates with underlying mutations. Cerebral convexity and most spinal meningiomas carry a 22q deletion and/or NF2 mutations, while skull base meningiomas have AKT1, TRAF7, SMO, and/or PIK3CA mutations. MRI is the primary imaging technique for diagnosing and treatment-planning of meningiomas, while DOTATATE PET imaging offers supplementary information beyond anatomic imaging. Herein, we review the evolving molecular landscape of meningiomas, emphasizing imaging/genetic biomarkers and treatment strategies relevant to neuroradiologists.

ABBREVIATIONS:

AKT1
AKT serine/threonine kinase 1
BAP1
BRCA1-associated protein 1
CDK4/6
cyclin-dependent kinases 4 and 6
CDKN2A/B
cyclin-dependent kinase inhibitor 2A/B
CNS5
Classification of Central Nervous System Tumors, fifth edition
KLF4
Krüppel-like factor 4
mTOR
mammalian target of rapamycin
NF2
neurofibromatosis type 2
PIK3CA
phosphatidylinositol-4,5-Bisphosphate 3-Kinase catalytic subunit alpha
POLR2A
RNA polymerase II subunit A
pTERT
telomerase reverse transcriptase promoter
SMARCB1
SWItch/sucrose non-fermentable related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1
SMO
smoothened, frizzled class receptor
SM
spinal meningioma
SUVmax
maximum standard uptake value
TERT
telomerase reverse transcriptase
TRAF7
tumor necrosis factor receptor–associated factor 7
WHO
World Health Organization
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American Journal of Neuroradiology: 46 (2)
American Journal of Neuroradiology
Vol. 46, Issue 2
1 Feb 2025
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Cite this article
Neetu Soni, Manish Ora, Girish Bathla, Denes Szekeres, Amit Desai, Jay J. Pillai, Amit Agarwal
Meningioma: Molecular Updates from the 2021 World Health Organization Classification of CNS Tumors and Imaging Correlates
American Journal of Neuroradiology Feb 2025, 46 (2) 240-250; DOI: 10.3174/ajnr.A8368

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Meningioma Updates from 2021 WHO Classification
Neetu Soni, Manish Ora, Girish Bathla, Denes Szekeres, Amit Desai, Jay J. Pillai, Amit Agarwal
American Journal of Neuroradiology Feb 2025, 46 (2) 240-250; DOI: 10.3174/ajnr.A8368
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