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Atypical and malignant meningiomas

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


Meningiomas account for approximately one-third of primary central nervous system tumors (table 1 and figure 1). Most meningiomas are benign (WHO grade I), although up to one-fifth of such tumors are classified as atypical (World Health Organization [WHO] grade II) or malignant (WHO grade III). (See "Meningioma: Epidemiology, risk factors, and pathology", section on 'Pathology'.)

The management of patients with meningioma requires a balance between definitive treatment of the tumor and avoidance of neurologic damage from the treatment. Patient-specific factors (eg, presence or absence of symptoms, age, comorbidity), the location of the meningioma in relation to critical brain structures and regions, and the histopathologic characteristics (WHO grade) of the meningioma all are important factors in determining the optimal treatment.

WHO grade II and grade III meningiomas will be reviewed here. Other topics on meningioma include:

Epidemiology and risk factors (see "Meningioma: Epidemiology, risk factors, and pathology")

Clinical presentation (see "Meningioma: Clinical presentation and diagnosis")


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Literature review current through: Jun 2016. | This topic last updated: Jan 6, 2016.
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  1. Perry, A, Louis, DN, Scheithauer, BW, et, al. Meningiomas. In: WHO Classification of Tumours of the Central Nervous System, Louis, DN, Ohgaki, H, Wiestler, OD (Eds), IARC Press, Lyon 2007. p.164.
  2. Hanft S, Canoll P, Bruce JN. A review of malignant meningiomas: diagnosis, characteristics, and treatment. J Neurooncol 2010; 99:433.
  3. Giannini, C, Rushing, EJ, Hainfellner, JA. Haemangiopericytoma. In: WHO Classification of Tumours of the Central Nervous System, Louis, DN, Ohgaki, H, Wiestler, OD, Cavenee, WK (Eds), IARC Press, Lyon 2007. p.178.
  4. 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.
  5. Zhang H, Rödiger LA, Shen T, et al. Perfusion MR imaging for differentiation of benign and malignant meningiomas. Neuroradiology 2008; 50:525.
  6. 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.
  7. Rogers L, Gilbert M, Vogelbaum MA. Intracranial meningiomas of atypical (WHO grade II) histology. J Neurooncol 2010; 99:393.
  8. Aizer AA, Bi WL, Kandola MS, et al. Extent of resection and overall survival for patients with atypical and malignant meningioma. Cancer 2015; 121:4376.
  9. Harris AE, Lee JY, Omalu B, et al. The effect of radiosurgery during management of aggressive meningiomas. Surg Neurol 2003; 60:298.
  10. Huffmann BC, Reinacher PC, Gilsbach JM. Gamma knife surgery for atypical meningiomas. J Neurosurg 2005; 102 Suppl:283.
  11. Kano H, Takahashi JA, Katsuki T, et al. Stereotactic radiosurgery for atypical and anaplastic meningiomas. J Neurooncol 2007; 84:41.
  12. Girvigian MR, Chen JC, Rahimian J, et al. Comparison of early complications for patients with convexity and parasagittal meningiomas treated with either stereotactic radiosurgery or fractionated stereotactic radiotherapy. Neurosurgery 2008; 62:A19.
  13. Lominska CE, Unger K, Jean WC, et al. Multisession stereotactic radiosurgery for meningioma results in low rates of post-treatment edema. Int J Radiat Oncol Biol Phys 2009. (Available online at http://astro2009.abstractsnet.com/pdfs/2135.pdfaccessed June 19, 2012).
  14. Boskos C, Feuvret L, Noel G, et al. Combined proton and photon conformal radiotherapy for intracranial atypical and malignant meningioma. Int J Radiat Oncol Biol Phys 2009; 75:399.
  15. Combs SE, Hartmann C, Nikoghosyan A, et al. Carbon ion radiation therapy for high-risk meningiomas. Radiother Oncol 2010; 95:54.
  16. Milosevic MF, Frost PJ, Laperriere NJ, et al. Radiotherapy for atypical or malignant intracranial meningioma. Int J Radiat Oncol Biol Phys 1996; 34:817.
  17. Dziuk TW, Woo S, Butler EB, et al. Malignant meningioma: an indication for initial aggressive surgery and adjuvant radiotherapy. J Neurooncol 1998; 37:177.
  18. Rosenberg LA, Prayson RA, Lee J, et al. Long-term experience with World Health Organization grade III (malignant) meningiomas at a single institution. Int J Radiat Oncol Biol Phys 2009; 74:427.
  19. Sughrue ME, Sanai N, Shangari G, et al. Outcome and survival following primary and repeat surgery for World Health Organization Grade III meningiomas. J Neurosurg 2010; 113:202.
  20. Perry A, Scheithauer BW, Stafford SL, et al. "Malignancy" in meningiomas: a clinicopathologic study of 116 patients, with grading implications. Cancer 1999; 85:2046.
  21. 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.
  22. Hug EB, Devries A, Thornton AF, et al. Management of atypical and malignant meningiomas: role of high-dose, 3D-conformal radiation therapy. J Neurooncol 2000; 48:151.
  23. Chan AW, Bernstein KD, Adams JA, et al. Dose escalation with proton radiation therapy for high-grade meningiomas. Technol Cancer Res Treat 2012; 11:607.
  24. Aghi MK, Carter BS, Cosgrove GR, et al. Long-term recurrence rates of atypical meningiomas after gross total resection with or without postoperative adjuvant radiation. Neurosurgery 2009; 64:56.
  25. Komotar RJ, Iorgulescu JB, Raper DM, et al. The role of radiotherapy following gross-total resection of atypical meningiomas. J Neurosurg 2012; 117:679.
  26. Adeberg S, Hartmann C, Welzel T, et al. Long-term outcome after radiotherapy in patients with atypical and malignant meningiomas--clinical results in 85 patients treated in a single institution leading to optimized guidelines for early radiation therapy. Int J Radiat Oncol Biol Phys 2012; 83:859.
  27. Aizer AA, Arvold ND, Catalano P, et al. Adjuvant radiation therapy, local recurrence, and the need for salvage therapy in atypical meningioma. Neuro Oncol 2014; 16:1547.
  28. Park H, Kim I, Jung H. Aytpical meningioma: Outcomes and prognostic factors. Int J Radiat Oncol Biol Phys 2009; 75:S238.
  29. Hardesty DA, Wolf AB, Brachman DG, et al. The impact of adjuvant stereotactic radiosurgery on atypical meningioma recurrence following aggressive microsurgical resection. J Neurosurg 2013; 119:475.
  30. Yang SY, Park CK, Park SH, et al. Atypical and anaplastic meningiomas: prognostic implications of clinicopathological features. J Neurol Neurosurg Psychiatry 2008; 79:574.
  31. 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.
  32. Modha A, Gutin PH. Diagnosis and treatment of atypical and anaplastic meningiomas: a review. Neurosurgery 2005; 57:538.
  33. Park HJ, Kang HC, Kim IH, et al. The role of adjuvant radiotherapy in atypical meningioma. J Neurooncol 2013; 115:241.
  34. Kaur G, Sayegh ET, Larson A, et al. Adjuvant radiotherapy for atypical and malignant meningiomas: a systematic review. Neuro Oncol 2014; 16:628.
  35. Mair R, Morris K, Scott I, Carroll TA. Radiotherapy for atypical meningiomas. J Neurosurg 2011; 115:811.
  36. ClinicalTrials.gov http://www.clinicaltrials.gov/ (Accessed on October 21, 2010).
  37. National Comprehensive Cancer Network (NCCN). NCCN Clinical practice guidelines in oncology. http://www.nccn.org/professionals/physician_gls/f_guidelines.asp (Accessed on February 27, 2016).
  38. Chuang HC, Lee HC, Cho DY. Intracranial malignant meningioma with multiple spinal metastases--a case report and literature review: case report. Spine (Phila Pa 1976) 2006; 31:E1006.
  39. Chamberlain MC, Glantz MJ. Cerebrospinal fluid-disseminated meningioma. Cancer 2005; 103:1427.
  40. Slavin ML. Metastatic malignant meningioma. J Clin Neuroophthalmol 1989; 9:55.
  41. Figueroa BE, Quint DJ, McKeever PE, Chandler WF. Extracranial metastatic meningioma. Br J Radiol 1999; 72:513.
  42. Combs SE, Schulz-Ertner D, Debus J, et al. Improved correlation of the neuropathologic classification according to adapted world health organization classification and outcome after radiotherapy in patients with atypical and anaplastic meningiomas. Int J Radiat Oncol Biol Phys 2011; 81:1415.
  43. Stessin AM, Schwartz A, Judanin G, et al. Does adjuvant external-beam radiotherapy improve outcomes for nonbenign meningiomas? A Surveillance, Epidemiology, and End Results (SEER)-based analysis. J Neurosurg 2012; 117:669.