RT Journal Article
SR Electronic
T1 Meningioma: Molecular Updates from the 2021 World Health Organization Classification of CNS Tumors and Imaging Correlates
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 240
OP 250
DO 10.3174/ajnr.A8368
VO 46
IS 2
A1 Soni, Neetu
A1 Ora, Manish
A1 Bathla, Girish
A1 Szekeres, Denes
A1 Desai, Amit
A1 Pillai, Jay J.
A1 Agarwal, Amit
YR 2025
UL http://www.ajnr.org/content/46/2/240.abstract
AB 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.AKT1AKT serine/threonine kinase 1BAP1BRCA1-associated protein 1CDK4/6cyclin-dependent kinases 4 and 6CDKN2A/Bcyclin-dependent kinase inhibitor 2A/BCNS5Classification of Central Nervous System Tumors, fifth editionKLF4Krüppel-like factor 4mTORmammalian target of rapamycinNF2neurofibromatosis type 2PIK3CAphosphatidylinositol-4,5-Bisphosphate 3-Kinase catalytic subunit alphaPOLR2ARNA polymerase II subunit ApTERTtelomerase reverse transcriptase promoterSMARCB1SWItch/sucrose non-fermentable related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1SMOsmoothened, frizzled class receptorSMspinal meningiomaSUVmaxmaximum standard uptake valueTERTtelomerase reverse transcriptaseTRAF7tumor necrosis factor receptor–associated factor 7WHOWorld Health Organization