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Epidemiology, pathology, clinical features, and diagnosis of meningioma

John K Park, MD, PhD
Section Editors
Jay S Loeffler, MD
Patrick Y Wen, MD
Deputy Editor
April F Eichler, MD, MPH


Meningiomas are the most frequent primary brain tumors (table 1 and figure 1). Although most meningiomas are benign, their location in the central nervous system can cause serious morbidity or mortality. (See "Incidence of primary brain tumors".)

The epidemiology, pathology, clinical presentation, and diagnosis of meningiomas will be reviewed here. Other topics on meningioma include:

Treatment of WHO grade I (benign) meningiomas (see "Management of known or presumed benign (WHO grade I) meningioma")

Treatment of WHO grade II and III meningiomas (see "Management of atypical and malignant (WHO grade II and III) meningioma")

Systemic therapy for recurrent meningioma (see "Systemic treatment of recurrent meningioma")

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Literature review current through: Nov 2017. | This topic last updated: Aug 08, 2017.
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  1. Wiemels J, Wrensch M, Claus EB. Epidemiology and etiology of meningioma. J Neurooncol 2010; 99:307.
  2. Central Brain Tumor Registry of the United States. Reports and tables 2010. http://www.cbtrus.org/reports/reports.html (Accessed on October 12, 2010).
  3. 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 4:iv1.
  4. Liu Y, Li F, Zhu S, et al. Clinical features and treatment of meningiomas in children: report of 12 cases and literature review. Pediatr Neurosurg 2008; 44:112.
  5. Marosi C, Hassler M, Roessler K, et al. Meningioma. Crit Rev Oncol Hematol 2008; 67:153.
  6. Banerjee J, Pääkkö E, Harila M, et al. Radiation-induced meningiomas: a shadow in the success story of childhood leukemia. Neuro Oncol 2009; 11:543.
  7. Claus EB, Bondy ML, Schildkraut JM, et al. Epidemiology of intracranial meningioma. Neurosurgery 2005; 57:1088.
  8. Umansky F, Shoshan Y, Rosenthal G, et al. Radiation-induced meningioma. Neurosurg Focus 2008; 24:E7.
  9. Kshettry VR, Ostrom QT, Kruchko C, et al. Descriptive epidemiology of World Health Organization grades II and III intracranial meningiomas in the United States. Neuro Oncol 2015; 17:1166.
  10. Willis J, Smith C, Ironside JW, et al. The accuracy of meningioma grading: a 10-year retrospective audit. Neuropathol Appl Neurobiol 2005; 31:141.
  11. Pearson BE, Markert JM, Fisher WS, et al. Hitting a moving target: evolution of a treatment paradigm for atypical meningiomas amid changing diagnostic criteria. Neurosurg Focus 2008; 24:E3.
  12. Braganza MZ, Kitahara CM, Berrington de González A, et al. Ionizing radiation and the risk of brain and central nervous system tumors: a systematic review. Neuro Oncol 2012; 14:1316.
  13. Friedman DL, Whitton J, Leisenring W, et al. Subsequent neoplasms in 5-year survivors of childhood cancer: the Childhood Cancer Survivor Study. J Natl Cancer Inst 2010; 102:1083.
  14. Taylor AJ, Little MP, Winter DL, et al. Population-based risks of CNS tumors in survivors of childhood cancer: the British Childhood Cancer Survivor Study. J Clin Oncol 2010; 28:5287.
  15. Bowers DC, Moskowitz CS, Chou JF, et al. Morbidity and Mortality Associated With Meningioma After Cranial Radiotherapy: A Report From the Childhood Cancer Survivor Study. J Clin Oncol 2017; 35:1570.
  16. Ron E, Modan B, Boice JD Jr, et al. Tumors of the brain and nervous system after radiotherapy in childhood. N Engl J Med 1988; 319:1033.
  17. Preston-Martin S, Yu MC, Henderson BE, Roberts C. Risk factors for meningiomas in men in Los Angeles County. J Natl Cancer Inst 1983; 70:863.
  18. Preston-Martin S, Mack W, Henderson BE. Risk factors for gliomas and meningiomas in males in Los Angeles County. Cancer Res 1989; 49:6137.
  19. Ryan P, Lee MW, North B, McMichael AJ. Amalgam fillings, diagnostic dental x-rays and tumours of the brain and meninges. Eur J Cancer B Oral Oncol 1992; 28B:91.
  20. Longstreth WT Jr, Phillips LE, Drangsholt M, et al. Dental X-rays and the risk of intracranial meningioma: a population-based case-control study. Cancer 2004; 100:1026.
  21. Claus EB, Calvocoressi L, Bondy ML, et al. Dental x-rays and risk of meningioma. Cancer 2012; 118:4530.
  22. Lin MC, Lee CF, Lin CL, et al. Dental diagnostic X-ray exposure and risk of benign and malignant brain tumors. Ann Oncol 2013; 24:1675.
  23. Preston-Martin S, Paganini-Hill A, Henderson BE, et al. Case-control study of intracranial meningiomas in women in Los Angeles County, California. J Natl Cancer Inst 1980; 65:67.
  24. Calnon WR. Shortcomings of study on dental x-rays and risk of meningioma. Cancer 2013; 119:464.
  25. Pearce MS, Salotti JA, Little MP, et al. Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet 2012; 380:499.
  26. Davis F, Il'yasova D, Rankin K, et al. Medical diagnostic radiation exposures and risk of gliomas. Radiat Res 2011; 175:790.
  27. Grant IS. Necrotising fasciitis. Lancet 1994; 344:1770.
  28. Plyusnin A, Vapalahti O, Vasilenko V, et al. Dobrava hantavirus in Estonia: does the virus exist throughout Europe? Lancet 1997; 349:1369.
  29. Preston DL, Ron E, Yonehara S, et al. Tumors of the nervous system and pituitary gland associated with atomic bomb radiation exposure. J Natl Cancer Inst 2002; 94:1555.
  30. Asgharian B, Chen YJ, Patronas NJ, et al. Meningiomas may be a component tumor of multiple endocrine neoplasia type 1. Clin Cancer Res 2004; 10:869.
  31. Goutagny S, Kalamarides M. Meningiomas and neurofibromatosis. J Neurooncol 2010; 99:341.
  32. Evans DG, Huson SM, Donnai D, et al. A clinical study of type 2 neurofibromatosis. Q J Med 1992; 84:603.
  33. Perry A, Giannini C, Raghavan R, et al. Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: a clinicopathologic study of 53 cases. J Neuropathol Exp Neurol 2001; 60:994.
  34. Larson JJ, van Loveren HR, Balko MG, Tew JM Jr. Evidence of meningioma infiltration into cranial nerves: clinical implications for cavernous sinus meningiomas. J Neurosurg 1995; 83:596.
  35. Carroll RS, Zhang J, Dashner K, et al. Androgen receptor expression in meningiomas. J Neurosurg 1995; 82:453.
  36. Blankenstein MA, Verheijen FM, Jacobs JM, et al. Occurrence, regulation, and significance of progesterone receptors in human meningioma. Steroids 2000; 65:795.
  37. Carroll RS, Zhang J, Black PM. Expression of estrogen receptors alpha and beta in human meningiomas. J Neurooncol 1999; 42:109.
  38. Jhawar BS, Fuchs CS, Colditz GA, Stampfer MJ. Sex steroid hormone exposures and risk for meningioma. J Neurosurg 2003; 99:848.
  39. Wigertz A, Lönn S, Mathiesen T, et al. Risk of brain tumors associated with exposure to exogenous female sex hormones. Am J Epidemiol 2006; 164:629.
  40. Blitshteyn S, Crook JE, Jaeckle KA. Is there an association between meningioma and hormone replacement therapy? J Clin Oncol 2008; 26:279.
  41. Benson VS, Pirie K, Green J, et al. Lifestyle factors and primary glioma and meningioma tumours in the Million Women Study cohort. Br J Cancer 2008; 99:185.
  42. Lee E, Grutsch J, Persky V, et al. Association of meningioma with reproductive factors. Int J Cancer 2006; 119:1152.
  43. Claus EB, Calvocoressi L, Bondy ML, et al. Exogenous hormone use, reproductive factors, and risk of intracranial meningioma in females. J Neurosurg 2013; 118:649.
  44. Benson VS, Kirichek O, Beral V, Green J. Menopausal hormone therapy and central nervous system tumor risk: large UK prospective study and meta-analysis. Int J Cancer 2015; 136:2369.
  45. Custer BS, Koepsell TD, Mueller BA. The association between breast carcinoma and meningioma in women. Cancer 2002; 94:1626.
  46. Johnson DR, Olson JE, Vierkant RA, et al. Risk factors for meningioma in postmenopausal women: results from the Iowa Women's Health Study. Neuro Oncol 2011; 13:1011.
  47. Michaud DS, Bové G, Gallo V, et al. Anthropometric measures, physical activity, and risk of glioma and meningioma in a large prospective cohort study. Cancer Prev Res (Phila) 2011; 4:1385.
  48. Wiedmann M, Brunborg C, Lindemann K, et al. Body mass index and the risk of meningioma, glioma and schwannoma in a large prospective cohort study (The HUNT Study). Br J Cancer 2013; 109:289.
  49. Schildkraut JM, Calvocoressi L, Wang F, et al. Endogenous and exogenous hormone exposure and the risk of meningioma in men. J Neurosurg 2014; 120:820.
  50. Niedermaier T, Behrens G, Schmid D, et al. Body mass index, physical activity, and risk of adult meningioma and glioma: A meta-analysis. Neurology 2015; 85:1342.
  51. Lauby-Secretan B, Scoccianti C, Loomis D, et al. Body Fatness and Cancer--Viewpoint of the IARC Working Group. N Engl J Med 2016; 375:794.
  52. Preston-Martin S, Pogoda JM, Schlehofer B, et al. An international case-control study of adult glioma and meningioma: the role of head trauma. Int J Epidemiol 1998; 27:579.
  53. Inskip PD, Mellemkjaer L, Gridley G, Olsen JH. Incidence of intracranial tumors following hospitalization for head injuries (Denmark). Cancer Causes Control 1998; 9:109.
  54. Preston-Martin S, Henderson BE, Yu MC. Epidemiology of intracranial meningiomas: Los Angeles Country, California. Neuroepidemiology 1983; 2:164.
  55. 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; 131:803.
  56. WHO Classification of Tumours of the Central Nervous System, 4th ed, Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds), IARC, Lyon 2016.
  57. Perry, A, Louis, et al. Meningiomas. In: WHO Classification of Tumours of the Central Nervous System, Louis, DN, Ohgaki, H, Wiestler, OD, Cavenee, WK (Eds), IARC Press, Lyon 2007. p.164.
  58. Yang SY, Park CK, Park SH, et al. Atypical and anaplastic meningiomas: prognostic implications of clinicopathological features. J Neurol Neurosurg Psychiatry 2008; 79:574.
  59. Pasquier D, Bijmolt S, Veninga T, et al. Atypical and malignant meningioma: outcome and prognostic factors in 119 irradiated patients. A multicenter, retrospective study of the Rare Cancer Network. Int J Radiat Oncol Biol Phys 2008; 71:1388.
  60. Palma L, Celli P, Franco C, et al. Long-term prognosis for atypical and malignant meningiomas: a study of 71 surgical cases. J Neurosurg 1997; 86:793.
  61. Perry A, Scheithauer BW, Stafford SL, et al. "Malignancy" in meningiomas: a clinicopathologic study of 116 patients, with grading implications. Cancer 1999; 85:2046.
  62. Hanft S, Canoll P, Bruce JN. A review of malignant meningiomas: diagnosis, characteristics, and treatment. J Neurooncol 2010; 99:433.
  63. Marciscano AE, Stemmer-Rachamimov AO, Niemierko A, et al. Benign meningiomas (WHO Grade I) with atypical histological features: correlation of histopathological features with clinical outcomes. J Neurosurg 2016; 124:106.
  64. Sahm F, Schrimpf D, Stichel D, et al. DNA methylation-based classification and grading system for meningioma: a multicentre, retrospective analysis. Lancet Oncol 2017; 18:682.
  65. Choy W, Kim W, Nagasawa D, et al. The molecular genetics and tumor pathogenesis of meningiomas and the future directions of meningioma treatments. Neurosurg Focus 2011; 30:E6.
  66. Brastianos PK, Horowitz PM, Santagata S, et al. Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations. Nat Genet 2013; 45:285.
  67. Clark VE, Erson-Omay EZ, Serin A, et al. Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO. Science 2013; 339:1077.
  68. Abedalthagafi M, Bi WL, Aizer AA, et al. Oncogenic PI3K mutations are as common as AKT1 and SMO mutations in meningioma. Neuro Oncol 2016; 18:649.
  69. Strickland MR, Gill CM, Nayyar N, et al. Targeted sequencing of SMO and AKT1 in anterior skull base meningiomas. J Neurosurg 2017; 127:438.
  70. Pachow D, Andrae N, Kliese N, et al. mTORC1 inhibitors suppress meningioma growth in mouse models. Clin Cancer Res 2013; 19:1180.
  71. Whittle IR, Smith C, Navoo P, Collie D. Meningiomas. Lancet 2004; 363:1535.
  72. Mattox A, Hughes B, Oleson J, et al. Treatment recommendations for primary extradural meningiomas. Cancer 2011; 117:24.
  73. Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med 2007; 357:1821.
  74. Annegers JF, Schoenberg BS, Okazaki H, Kurland LT. Epidemiologic study of primary intracranial neoplasms. Arch Neurol 1981; 38:217.
  75. Nakasu S, Hirano A, Shimura T, Llena JF. Incidental meningiomas in autopsy study. Surg Neurol 1987; 27:319.
  76. Niiro M, Yatsushiro K, Nakamura K, et al. Natural history of elderly patients with asymptomatic meningiomas. J Neurol Neurosurg Psychiatry 2000; 68:25.
  77. Go RS, Taylor BV, Kimmel DW. The natural history of asymptomatic meningiomas in Olmsted County, Minnesota. Neurology 1998; 51:1718.
  78. Morris Z, Whiteley WN, Longstreth WT Jr, et al. Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. BMJ 2009; 339:b3016.
  79. Bos D, Poels MM, Adams HH, et al. Prevalence, Clinical Management, and Natural Course of Incidental Findings on Brain MR Images: The Population-based Rotterdam Scan Study. Radiology 2016; 281:507.
  80. Englot DJ, Magill ST, Han SJ, et al. Seizures in supratentorial meningioma: a systematic review and meta-analysis. J Neurosurg 2016; 124:1552.
  81. Pieper DR, Al-Mefty O, Hanada Y, Buechner D. Hyperostosis associated with meningioma of the cranial base: secondary changes or tumor invasion. Neurosurgery 1999; 44:742.
  82. Hsu CC, Pai CY, Kao HW, et al. Do aggressive imaging features correlate with advanced histopathological grade in meningiomas? J Clin Neurosci 2010; 17:584.
  83. Zhang H, Rödiger LA, Shen T, et al. Perfusion MR imaging for differentiation of benign and malignant meningiomas. Neuroradiology 2008; 50:525.
  84. Nagar VA, Ye JR, Ng WH, et al. Diffusion-weighted MR imaging: diagnosing atypical or malignant meningiomas and detecting tumor dedifferentiation. AJNR Am J Neuroradiol 2008; 29:1147.
  85. Rogers L, Gilbert M, Vogelbaum MA. Intracranial meningiomas of atypical (WHO grade II) histology. J Neurooncol 2010; 99:393.
  86. Johnson MD, Powell SZ, Boyer PJ, et al. Dural lesions mimicking meningiomas. Hum Pathol 2002; 33:1211.
  87. Tu PH, Giannini C, Judkins AR, et al. Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: a primary low-grade CNS lymphoma that mimics meningioma. J Clin Oncol 2005; 23:5718.
  88. Tan LA, Kasliwal MK, Wewel J, et al. Neurosarcoidosis mimicking bilateral posterior fossa tentorial meningiomas. J Neurooncol 2015; 125:435.