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meningioma

Generally benign tumor of meningial origin. Classification: 1) benign meningioma (WHO grade I) [7] 2) atypical meningioma (WHO grade 2) 3) anaplastic meningioma, rhabdoid meningioma (WHO grade 3) Etiology: - exposure to exogenous androgens, estrogens &/or progestins may be risk factor [2] - overweight & lack of exercise are associated with increased likelihood of meningioma (RR=1.2-1.5) [5] Epidemiology: 1) ~13-19% of intracranial tumors (most common brain tumor) 2) ~25% of intraspinal neoplasms 3) peak incidence in middle age 4) more frequent in women than men 5) most patients 50-65 years of age [3] Pathology: 1) composed of neoplastic meningothelial or arachnoid cells 2) attaches to dura 3) may invade skull, but almost never invade brain 4) slow growth, averaging 0.24 cm in diameter/year 5) frequently occur: a) along sagittal sinus b) over cerebral convexities c) in cerebellar-pontine angle d) along dorsum of spinal cord 6) most meningiomas are estrogen receptor & progesterone receptor positive 7) 50% of meningiomas are androgen receptor positive [3] Microscopic pathology: 1) variable appearances/subtypes: a) meningothelial b) fibrous c) transitional (mixed) d) psammomatous e) angiomatous f) microcystic g) secretory h) lymphoplasmacyte-rich i) metaplastic j) clear cell k) chordoid 2) variants a-i correspond to WHO grade I, j & k correspond to grade II with increased rate of recurrence or aggressive behavior Immunohistochemistry: - EMA + - vimentin + - S100 variable, usually not prominant - CEA + in secretory meningiomas Genetics: see meningeal neoplasm Clinical manifestations: 1) may be asymptomatic incidental finding on CT or MRI 2) patients rarely become symptomatic a) seizures b) focal neurologic deficit c) signs of increased intracranial pressure - progressive headache Radiology: 1) computed tomography (CT) with contrast a) partially calcified, homogenously enhancing extra-axial mass adherent to dura b) enhancing dural 'tail' 2) magnetic resonance imaging (MRI) with gadolinium contrast - homogeneous contrast enhancement 'light bulb sign' - diffusely enhancing - case describing incidental parafalcine hyperintensity [3] - enhancing dural 'tail' [3] * image [7] Management: 1) small asymptomatic meningiomas in older adults may be managed by observation [3,6] a) follow-up imaging b) regular clinical evaluation 2) surgical resection a) symptomatic meningiomas - drug-resistance seizures [3] - severe headaches b) enlarging meningiomas c) perineoplastic edema d) younger patients [3] 3) arterial embolization [3] 4) radiation therapy for residual tumor - lutetium-177 dotatate (Lutathera) extends progression-free survival in patients with radiation-refractory intracranial meningioma [9] 5) chemotherapy of no benefit [3] Prognosis: 1) small asymptomatic meningiomas may be observed - initial repeat MRI in 3-6 months [3] 2) surgical excision often curative 3) radiation therapy reduces recurrence to < 10% 4) life expectancy: 3 years to normal life expectancy depending upon grade of neoplasm [3]

Related

hemangiopericytoma of meninges

Specific

anaplastic meningioma; malignant meningioma atypical meningioma papillary meningioma rhabdoid meningioma

General

meningeal neoplasm

References

  1. WHO Classification Tumours of the Nervous System. Kleihues & Cavenee eds. IARC Press 2000
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2398
  3. Medical Knowledge Self Assessment Program (MKSAP) 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2006, 2009, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. Whittle IR, Smith C, Navoo P, Collie D. Meningiomas. Lancet. 2004 May 8;363(9420):1535-43. PMID: 15135603
  5. Niedermaier T et al Body mass index, physical activity, and risk of adult meningioma and glioma. Neurology. September 16, 2015 PMID: 26377253 http://www.neurology.org/content/early/2015/09/16/WNL.0000000000002020
  6. Yano S, Kuratsu J; Kumamoto Brain Tumor Research Group. Indications for surgery in patients with asymptomatic meningiomas based on an extensive experience. J Neurosurg. 2006 Oct;105(4):538-43. PMID: 17044555
  7. Khanna O, Ghobrial GM, Farrell CJ 10 Brain Lesions to Recognize (MRI images) Medscape. October 25, 2021 https://reference.medscape.com/slideshow/brain-lesions-6013313
  8. Wiemels J, Wrensch M, Claus EB. Epidemiology and etiology of meningioma J Neurooncol. 2010 Sep;99(3):307-14. PMID: 20821343 PMCID: PMC2945461 Free PMC article
  9. Bassett M 'Significant Milestone' for Radionuclide Therapy in Refractory Meningioma Small study suggests large benefit for Lu-177 dotatate in intracranial disease. MedPage Today October 2, 2024 https://www.medpagetoday.com/meetingcoverage/astro/112234

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OMIM correlations images related to meningioma