Pathologic diagnosis
The World Health Organization (WHO) has produced guidelines for grading.[2]World Health Organization Classification of Tumours Editorial Board. WHO classification of tumours of the central nervous system. 5th Edition. France: IARC; 2021.[3]Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.
https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/34185076
http://www.ncbi.nlm.nih.gov/pubmed/34185076?tool=bestpractice.com
There are 3 grades of meningioma: 1, 2, and 3. The vast majority of tumors are classified as WHO grade 1 (benign). WHO grade 2 tumors are referred to as atypical, while WHO grade 3 tumors are malignant meningiomas. See Classification.
The diagnostic criteria for WHO grade 2 and 3 tumors continues to evolve. In the 2007 update, there were changes with mitotic counts, which resulted in an increased prevalence of atypical meningiomas (from around 5% of cases, to 20% to 35% of all meningiomas).[4]Rogers L, Gilbert M, Vogelbaum MA. Intracranial meningiomas of atypical (WHO grade II) histology. J Neurooncol. 2010 Sep;99(3):393-405.
http://www.ncbi.nlm.nih.gov/pubmed/20740303?tool=bestpractice.com
[56]Louis DN, Ohgaki H, Westler OD, et al (eds). World Health Organization classification of tumours of the central nervous system. Lyon, France: IARC; 2007. In the 2016 update, brain invasion was added as a criteria for WHO grade 2 meningioma.[57]Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016 Jun;131(6):803-20.
https://link.springer.com/article/10.1007%2Fs00401-016-1545-1
http://www.ncbi.nlm.nih.gov/pubmed/27157931?tool=bestpractice.com
The 2021 update added molecular criteria for WHO grade 3 meningioma (homozygous CDKN2A/B loss or TERT promoter mutation). It also replaced the use of roman numerals (I, II, III) with arabic numerals (1, 2, 3).[3]Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.
https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/34185076
http://www.ncbi.nlm.nih.gov/pubmed/34185076?tool=bestpractice.com
The cIMPACT-NOW group has published a consensus on progress incorporating additional molecular criteria into the pathologic diagnosis, and highlighted situations in which additional molecular testing may be considered.[58]Sahm F, Aldape KD, Brastianos PK, et al. cIMPACT-NOW update 8: clarifications on molecular risk parameters and recommendations for WHO grading of meningiomas. Neuro Oncol. 2025 Feb 10;27(2):319-30.
https://academic.oup.com/neuro-oncology/article/27/2/319/7745776
http://www.ncbi.nlm.nih.gov/pubmed/39212325?tool=bestpractice.com
In particular, situations to consider molecular testing include tumors with borderline histologic criteria between WHO grade 1 and 2, brain-invasive but otherwise benign meningioma, tumors with rapid preoperative growth, and WHO grade 2 tumors.