Semin Neurol 2024; 44(01): 001-015
DOI: 10.1055/s-0043-1777352
Review Article

The Natural History and Treatment of Meningiomas: An Update

Arsene Daniel Nyalundja
1   Faculty of Medicine, Center for Tropical Diseases and Global Health, Université Catholique de Bukavu, Kadutu, Bukavu, South Kivu, Democratic Republique of Congo
,
Fabrice Mugisha
2   Neurosurgery Unit, Department of Surgery, Rwanda Military Hospital, Kigali, Rwanda
,
2   Neurosurgery Unit, Department of Surgery, Rwanda Military Hospital, Kigali, Rwanda
› Author Affiliations
Funding None.

Abstract

Meningiomas are the most frequent nonmalignant tumors of the central nervous system (CNS). Despite their benign nature and slow-growing pattern, if not diagnosed early, these tumors may reach relatively large sizes causing significant morbidity and mortality. Some variants are located in hard-to-access locations, compressing critical neurovascular structures, and making the surgical management even more challenging. Although most meningiomas have a good long-term prognosis after treatment, there are still controversies over their management in a subset of cases. While surgery is the first-line treatment, the use of fractionated radiotherapy or stereotactic radiosurgery is indicated for residual or recurrent tumors, small lesions, and tumors in challenging locations. Advances in molecular genetics and ongoing clinical trial results have recently helped both to refine the diagnosis and provide hope for effective biomolecular target-based medications for treatment. This article reviews the natural history and current therapeutic options for CNS meningiomas.



Publication History

Article published online:
05 December 2023

© 2023. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 
  • References

  • 1 Albayrak SB, Black PM. The origin of meningiomas. In: Pamir MN, Black PM, Fahlbusch R. eds. Meningiomas. W.B. Saunders; 2010: 53-60
  • 2 Kshettry VR, Ostrom QT, Kruchko C, Al-Mefty O, Barnett GH, Barnholtz-Sloan JS. Descriptive epidemiology of World Health Organization grades II and III intracranial meningiomas in the United States. Neuro-oncol 2015; 17 (08) 1166-1173
  • 3 Ostrom QT, Cioffi G, Gittleman H. et al. CBTRUS Statistical Report: primary brain and other central nervous system tumors diagnosed in the United States in 2012-2016. Neuro-oncol 2019; 21 (Suppl. 05) v1-v100
  • 4 Lim YS, Kim MK, Park BJ, Kim TS, Lim YJ. Long term clinical outcomes of malignant meningiomas. Brain Tumor Res Treat 2013; 1 (02) 85-90
  • 5 Wiemels J, Wrensch M, Claus EB. Epidemiology and etiology of meningioma. J Neurooncol 2010; 99 (03) 307-314
  • 6 Baldi I, Engelhardt J, Bonnet C. et al. Epidemiology of meningiomas. Neurochirurgie 2018; 64 (01) 5-14
  • 7 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; 131 (06) 803-820
  • 8 Louis DN, Perry A, Wesseling P. et al. The 2021 WHO Classification of tumors of the central nervous system: a summary. Neuro-oncol 2021; 23 (08) 1231-1251
  • 9 Harter PN, Braun Y, Plate KH. Classification of meningiomas-advances and controversies. Chin Clin Oncol 2017; 6 (Suppl. 01) S2-S2
  • 10 Sun C, Dou Z, Wu J. et al. The preferred locations of meningioma according to different biological characteristics based on voxel-wise analysis. Front Oncol 2020; 10: 1412
  • 11 Ostrom QT, Gittleman H, Fulop J. et al. CBTRUS Statistical Report: primary brain and central nervous system tumors diagnosed in the United States in 2008-2012. Neuro-oncol 2015; 17 (Suppl. 04) iv1-iv62
  • 12 Dziuk TW, Woo S, Butler EB. et al. Malignant meningioma: an indication for initial aggressive surgery and adjuvant radiotherapy. J Neurooncol 1998; 37 (02) 177-188
  • 13 Hammouche S, Clark S, Wong AHL, Eldridge P, Farah JO. Long-term survival analysis of atypical meningiomas: survival rates, prognostic factors, operative and radiotherapy treatment. Acta Neurochir (Wien) 2014; 156 (08) 1475-1481
  • 14 Kanmounye US, Karekezi C, Nyalundja AD, Awad AK, Laeke T, Balogun JA. Adult brain tumors in Sub-Saharan Africa: a scoping review. Neuro-oncol 2022; 24 (10) 1799-1806
  • 15 Meningiomas. Their classification, regional behaviour, life history, and surgical end results. Bull Med Libr Assoc 1938; 27 (02) 185
  • 16 Kurland LT, Schoenberg BS, Annegers JF, Okazaki H, Molgaard CA. The incidence of primary intracranial neoplasms in Rochester, Minnesota, 1935-1977. Ann N Y Acad Sci 1982; 381: 6-16
  • 17 Achey RL, Gittleman H, Schroer J, Khanna V, Kruchko C, Barnholtz-Sloan JS. Nonmalignant and malignant meningioma incidence and survival in the elderly, 2005-2015, using the Central Brain Tumor Registry of the United States. Neuro-oncol 2019; 21 (03) 380-391
  • 18 Ostrom QT, Gittleman H, Farah P. et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2006-2010. Neuro-oncol 2013; 15 (Suppl. 02) ii1-ii56
  • 19 Holleczek B, Zampella D, Urbschat S. et al. Incidence, mortality and outcome of meningiomas: a population-based study from Germany. Cancer Epidemiol 2019; 62: 101562
  • 20 Kang H, Song SW, Ha J. et al. A nationwide, population-based epidemiology study of primary central nervous system tumors in Korea, 2007-2016: a comparison with United States data. Cancer Res Treat 2021; 53 (02) 355-366
  • 21 Mbi Feh MKN, Lyon KA, Brahmaroutu AV, Tadipatri R, Fonkem E. The need for a central brain tumor registry in Africa: a review of central nervous system tumors in Africa from 1960 to 2017. Neurooncol Pract 2021; 8 (03) 337-344
  • 22 Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: primary brain and other central nervous system tumors diagnosed in the United States in 2011-2015. Neuro-oncol 2018; 20 (Suppl. 04) iv1-iv86
  • 23 Bachir S, Shah S, Shapiro S. et al. Neurofibromatosis Type 2 (NF2) and the implications for vestibular schwannoma and meningioma pathogenesis. Int J Mol Sci 2021; 22 (02) 690
  • 24 Goutagny S, Kalamarides M. Meningiomas and neurofibromatosis. J Neurooncol 2010; 99 (03) 341-347
  • 25 Aboukais R, Bonne NX, Baroncini M. et al. Management of multiple tumors in neurofibromatosis type 2 patients. Neurochirurgie 2018; 64 (05) 364-369
  • 26 Phillips D, Auguste KI, Gupta N. Meningiomas in children. In: McDermott MW. ed. Handbook of Clinical Neurology. Vol 169. Meningiomas, Part I. Elsevier; 2020: 253-259 DOI: 10.1016/B978-0-12-804280-9.00016-0
  • 27 Fan M-C, Fang W, Liu K. et al. Paediatric intracranial meningiomas: eight-year experience with 32 cases. Chin Neurosurg J 2017; 3 (01) 21
  • 28 Thevandiran D, Nga V, Chang KTE. et al. Paediatric meningiomas in Singapore - case series of a rare entity. J Clin Neurosci 2020; 73: 62-66
  • 29 Sadetzki S, Flint-Richter P, Starinsky S. et al. Genotyping of patients with sporadic and radiation-associated meningiomas. Cancer Epidemiol Biomarkers Prev 2005; 14 (04) 969-976
  • 30 Ron E, Modan B, Boice Jr JD. et al. Tumors of the brain and nervous system after radiotherapy in childhood. N Engl J Med 1988; 319 (16) 1033-1039
  • 31 Thompson DE, Mabuchi K, Ron E. et al. Cancer incidence in atomic bomb survivors. Part II: Solid tumors, 1958-1987. Radiat Res 1994; 137 (2, suppl): S17-S67
  • 32 Bowers DC, Nathan PC, Constine L. et al. Subsequent neoplasms of the CNS among survivors of childhood cancer: a systematic review. Lancet Oncol 2013; 14 (08) e321-e328
  • 33 Harrison MJ, Wolfe DE, Lau TS, Mitnick RJ, Sachdev VP. Radiation-induced meningiomas: experience at the Mount Sinai Hospital and review of the literature. J Neurosurg 1991; 75 (04) 564-574
  • 34 Rubinstein AB, Shalit MN, Cohen ML, Zandbank U, Reichenthal E. Radiation-induced cerebral meningioma: a recognizable entity. J Neurosurg 1984; 61 (05) 966-971
  • 35 Soffer D, Gomori JM, Siegal T, Shalit MN. Intracranial meningiomas after high-dose irradiation. Cancer 1989; 63 (08) 1514-1519
  • 36 Tabernero MD, Maillo A, Gil-Bellosta CJ. et al. Gene expression profiles of meningiomas are associated with tumor cytogenetics and patient outcome. Brain Pathol 2009; 19 (03) 409-420
  • 37 Ragel BT, Jensen RL. Molecular genetics of meningiomas. Neurosurg Focus 2005; 19 (05) E9
  • 38 Kalamarides M, Niwa-Kawakita M, Leblois H. et al. Nf2 gene inactivation in arachnoidal cells is rate-limiting for meningioma development in the mouse. Genes Dev 2002; 16 (09) 1060-1065
  • 39 Müller P, Henn W, Niedermayer I. et al. Deletion of chromosome 1p and loss of expression of alkaline phosphatase indicate progression of meningiomas. Clin Cancer Res 1999; 5 (11) 3569-3577
  • 40 Ketter R, Henn W, Niedermayer I. et al. Predictive value of progression-associated chromosomal aberrations for the prognosis of meningiomas: a retrospective study of 198 cases. J Neurosurg 2001; 95 (04) 601-607
  • 41 Karsy M, Azab MA, Abou-Al-Shaar H. et al. Clinical potential of meningioma genomic insights: a practical review for neurosurgeons. Neurosurg Focus 2018; 44 (06) E10
  • 42 Ogasawara C, Philbrick BD, Adamson DC. Meningioma: a review of epidemiology, pathology, diagnosis, treatment, and future directions. Biomedicines 2021; 9 (03) 319
  • 43 Lee YS, Lee YS. Molecular characteristics of meningiomas. J Pathol Transl Med 2020; 54 (01) 45-63
  • 44 Yakubov E, Ghoochani A, Buslei R, Buchfelder M, Eyüpoglu IY, Savaskan N. Hidden association of Cowden syndrome, PTEN mutation and meningioma frequency. Oncoscience 2016; 3 (5-6): 149-155
  • 45 Youngblood MW, Duran D, Montejo JD. et al. Correlations between genomic subgroup and clinical features in a cohort of more than 3000 meningiomas. J Neurosurg 2019; DOI: 10.3171/2019.8.JNS191266.
  • 46 Sahm F, Schrimpf D, Stichel D. et al. DNA methylation-based classification and grading system for meningioma: a multicentre, retrospective analysis. Lancet Oncol 2017; 18 (05) 682-694
  • 47 Driver J, Hoffman SE, Tavakol S. et al. A molecularly integrated grade for meningioma. Neuro-oncol 2022; 24 (05) 796-808
  • 48 Perry A, Stafford SL, Scheithauer BW, Suman VJ, Lohse CM. Meningioma grading: an analysis of histologic parameters. Am J Surg Pathol 1997; 21 (12) 1455-1465
  • 49 Nassiri F, Liu J, Patil V. et al. A clinically applicable integrative molecular classification of meningiomas. Nature 2021; 597 (7874): 119-125
  • 50 Goldbrunner R, Stavrinou P, Jenkinson MD. et al. EANO guideline on the diagnosis and management of meningiomas. Neuro-oncol 2021; 23 (11) 1821-1834
  • 51 Yamashita J, Handa H, Iwaki K, Abe M. Recurrence of intracranial meningiomas, with special reference to radiotherapy. Surg Neurol 1980; 14 (01) 33-40
  • 52 Goffaux P, Fortin D. Brain tumor headaches: from bedside to bench. Neurosurgery 2010; 67 (02) 459-466
  • 53 Zouaoui S, Darlix A, Rigau V. et al; French Brain Tumor DataBase (FBTDB) Participants and Investigators, with the participation of the Société française de neurochirurgie (SFNC), Club de neuro-oncologie de la SFNC, Société française de neuropathologie (SFNP), Association des neuro-oncologues d'expression française (ANOCEF). Descriptive epidemiology of 13,038 newly diagnosed and histologically confirmed meningiomas in France: 2006-2010. Neurochirurgie 2018; 64 (01) 15-21
  • 54 Elbadry Ahmed R, Tang H, Asemota A, Huang L, Boling W, Bannout F. Meningioma related epilepsy - pathophysiology, pre/postoperative seizures predicators and treatment. Front Oncol 2022; 12: 905976
  • 55 Hess K, Spille DC, Adeli A. et al. Brain invasion and the risk of seizures in patients with meningioma. J Neurosurg 2018; 130 (03) 789-796
  • 56 Alruwaili AA, De Jesus O. Meningioma. In: StatPearls. StatPearls Publishing; 2022. Accessed February 19, 2023 at: http://www.ncbi.nlm.nih.gov/books/NBK560538/
  • 57 Musa MJ, Zeppieri M. Foster Kennedy Syndrome. In: StatPearls.: StatPearls Publishing; 2022. Accessed February 25, 2023 at: http://www.ncbi.nlm.nih.gov/books/NBK582149/
  • 58 Samartzis D, Gillis CC, Shih P, O'Toole JE, Fessler RG. Intramedullary spinal cord tumors: Part I - Epidemiology, pathophysiology, and diagnosis. Global Spine J 2015; 5 (05) 425-435
  • 59 Huang RY, Bi WL, Griffith B. et al; International Consortium on Meningiomas. Imaging and diagnostic advances for intracranial meningiomas. Neuro-oncol 2019; 21 (Suppl. 01) i44-i61
  • 60 Lyndon D, Lansley JA, Evanson J, Krishnan AS. Dural masses: meningiomas and their mimics. Insights Imaging 2019; 10 (01) 11
  • 61 Hou J, Kshettry VR, Selman WR, Bambakidis NC. Peritumoral brain edema in intracranial meningiomas: the emergence of vascular endothelial growth factor-directed therapy. Neurosurg Focus 2013; 35 (06) E2
  • 62 Kim BW, Kim MS, Kim SW, Chang CH, Kim OL. Peritumoral brain edema in meningiomas: correlation of radiologic and pathologic features. J Korean Neurosurg Soc 2011; 49 (01) 26-30
  • 63 Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol 1990; 11 (05) 935-938
  • 64 Watts J, Box G, Galvin A, Brotchie P, Trost N, Sutherland T. Magnetic resonance imaging of meningiomas: a pictorial review. Insights Imaging 2014; 5 (01) 113-122
  • 65 Kessler RA, Garzon-Muvdi T, Yang W. et al. Metastatic atypical and anaplastic meningioma: a case series and review of the literature. World Neurosurg 2017; 101: 47-56
  • 66 Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural “tail” associated with meningiomas on Gd-DTPA-enhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment. Radiology 1990; 176 (02) 447-450
  • 67 Sotoudeh H, Yazdi HR. A review on dural tail sign. World J Radiol 2010; 2 (05) 188-192
  • 68 Tsuchiya K, Katase S, Yoshino A, Hachiya J. MR digital subtraction angiography in the diagnosis of meningiomas. Eur J Radiol 2003; 46 (02) 130-138
  • 69 Yin Y, Li Y, Jiang Z. et al. Clinical outcomes and complications of preoperative embolization for intracranial giant meningioma tumorectomy: a retrospective, observational, matched cohort study. Front Oncol 2022; 12: 852327
  • 70 Papacci F, Pedicelli A, Montano N. The role of preoperative angiography in the management of giant meningiomas associated to vascular malformation. Surg Neurol Int 2015; 6: 114
  • 71 Meling TR, Da Broi M, Scheie D, Helseth E, Smoll NR. Meningioma surgery - Are we making progress?. World Neurosurg 2019; 125: e205-e213
  • 72 NCCN Clinical Practice Guidelines in Oncology - Central Nervous System Cancers. National Comprehensive Cancer Network. Accessed February 27, 2023 at: https://www.nccn.org/professionals/physician_gls/pdf/cns.pdf
  • 73 Chotai S, Schwartz TH. The Simpson grading: Is it still valid?. Cancers (Basel) 2022; 14 (08) 2007
  • 74 Wong RH, Wong AK, Vick N, Farhat HI. Natural history of multiple meningiomas. Surg Neurol Int 2013; 4: 71
  • 75 Spille DC, Hess K, Bormann E. et al. Risk of tumor recurrence in intracranial meningiomas: comparative analyses of the predictive value of the postoperative tumor volume and the Simpson classification. J Neurosurg 2020; 134 (06) 1764-1771
  • 76 Voß KM, Spille DC, Sauerland C. et al. The Simpson grading in meningioma surgery: does the tumor location influence the prognostic value?. J Neurooncol 2017; 133 (03) 641-651
  • 77 Schwartz TH, McDermott MW. The Simpson grade: abandon the scale but preserve the message. J Neurosurg 2020; DOI: 10.3171/2020.6.JNS201904.
  • 78 El-Hajj VG, Pettersson-Segerlind J, Fletcher-Sandersjöö A, Edström E, Elmi-Terander A. Current knowledge on spinal meningiomas epidemiology, tumor characteristics and non-surgical treatment options: a systematic review and pooled analysis (Part 1). Cancers (Basel) 2022; 14 (24) 6251
  • 79 Wang N, Osswald M. Meningiomas: overview and new directions in therapy. Semin Neurol 2018; 38 (01) 112-120
  • 80 Mair MJ, Berghoff AS, Brastianos PK, Preusser M. Emerging systemic treatment options in meningioma. J Neurooncol 2023; 161 (02) 245-258
  • 81 Choudhury A, Magill ST, Eaton CD. et al. Meningioma DNA methylation groups identify biological drivers and therapeutic vulnerabilities. Nat Genet 2022; 54 (05) 649-659
  • 82 Lynes J, Flores-Milan G, Rubino S. et al. Molecular determinants of outcomes in meningiomas. Front Oncol 2022; 12: 962702
  • 83 Shahbandi A, Shah DS, Hadley CC, Patel AJ. The role of pharmacotherapy in treatment of meningioma: a systematic review. Cancers (Basel) 2023; 15 (02) 483
  • 84 Ibraheem A, Pillai C, Okoye I. et al. Cancer clinical trials in Africa - an untapped opportunity: recommendations from AORTIC 2019 conference special interest group in clinical trials. JCO Glob Oncol 2021; 7: 1358-1363
  • 85 Simpson D. The recurrence of intracranial meningiomas after surgical treatment. J Neurol Neurosurg Psychiatry 1957; 20 (01) 22-39
  • 86 Aizer AA, Bi WL, Kandola MS. et al. Extent of resection and overall survival for patients with atypical and malignant meningioma. Cancer 2015; 121 (24) 4376-4381
  • 87 Yuzawa S, Nishihara H, Tanaka S. Genetic landscape of meningioma. Brain Tumor Pathol 2016; 33 (04) 237-247
  • 88 Seliger C, Meier CR, Becker C. et al. Metabolic syndrome in relation to risk of meningioma. Oncotarget 2017; 8 (02) 2284-2292
  • 89 Meng Y, Tang C, Yu J, Meng S, Zhang W. Exposure to lead increases the risk of meningioma and brain cancer: a meta-analysis. J Trace Elem Med Biol 2020; 60: 126474
  • 90 Lee KS, Zhang JJY, Kirollos R, Santarius T, Nga VDW, Yeo TT. A systematic review and meta-analysis of the association between cyproterone acetate and intracranial meningiomas. Sci Rep 2022; 12 (01) 1942
  • 91 Nanda A, Bir SC, Maiti TK, Konar SK, Missios S, Guthikonda B. Relevance of Simpson grading system and recurrence-free survival after surgery for World Health Organization Grade I meningioma. J Neurosurg 2017; 126 (01) 201-211