Introduction
Background
Meningioma, the term coined by Harvey Cushing, refers to a set of tumors that arise contiguously to the meninges.
Pathophysiology
Meningiomas may occur intracranially or within the spinal canal. They are thought to arise from arachnoidal cap cells, which reside in the arachnoid layer covering the surface of the brain.
Meningiomas commonly are found at the surface of the brain, either over the convexity or at the skull base. In rare cases, meningiomas occur in an intraventricular or intraosseous location. The problem of classifying meningioma is that arachnoidal cells may express both mesenchymal and epithelial characteristics. Other mesodermal structures also may give rise to similar tumors (eg, hemangiopericytomas or sarcomas). The classification of all of these tumors together is controversial. The current trend is to separate unequivocal meningiomas from other less well-defined neoplasms. Undoubtedly, advances in molecular biology will allow scientists to determine the exact genomic aberration responsible for each specific neoplasm.
Frequency
United States
The annual incidence of symptomatic meningiomas is approximately 2 cases per 100,000 individuals. Meningiomas account for approximately 20% of all primary intracranial neoplasms. However, the true prevalence is likely higher than this because autopsy studies reveal that 2.3% of individuals have undiagnosed asymptomatic meningiomas. Meningiomas are multiple in 5-40% of cases, particularly when they associated with neurofibromatosis type 2 (NF2). Familial meningiomas are rare unless associated with NF2.1
International
The frequency of meningiomas in Africa is nearly 30% of all primary intracranial tumors.
Mortality/Morbidity
Mortality and morbidity rates for meningiomas are difficult to assess. Some meningiomas are discovered fortuitously when CT or MRI is done to assess for unrelated diseases or conditions. Therefore, some patients die with meningioma and not from it. Estimates of the 5-year survival usually range from 73-94%.
- Meningiomas usually grow slowly, and they may produce severe morbidity before causing death.
- Factors that may be predictive of a high postoperative morbidity rate include patient-related factors (eg, advanced age, comorbid states such as diabetes or coronary artery disease, preoperative neurological status), tumor factors (eg, location, size, consistency, vascularity, vascular or neural involvement), previous surgery, or previous radiation therapy.
Race
Meningiomas are more prevalent in Africa than in North America or Europe. In Los Angeles County, meningioma is reported more commonly in African Americans than in others.
Sex
Meningiomas afflict women more often than men. The male-to-female ratio ranges from 1:1.4 to 1:2.8.
- The female preponderance may be less pronounced in the black population than in other groups.
- Meningiomas are equally distributed between boys and girls.
Age
The incidence increases with age. Ages and corresponding incidence rates reported from 2002 are as follows:
- Age 0-19 years - 0.12
- Age 20-34 years - 0.74
- Age 35-44 years - 2.62
- Age 45-54 years - 4.89
- Age 55-64 years - 7.89
- Age 65-74 years - 12.79
- Age 75-84 years - 17.04
- Age 85 years and older - 18.86
Clinical
History
Meningiomas produce their symptoms by several mechanisms. They may cause symptoms by irritating the underlying cortex, compressing the brain or the cranial nerves, producing hyperostosis2 and/or invading the overlying soft tissues, or inducing vascular injuries to the brain. The signs and symptoms secondary to meningiomas may appear or become exacerbated during pregnancy but usually abate or improve in the postpartum period.
- Irritation: By irritating the underlying cortex, meningiomas can cause seizures. New-onset seizures in adults justify neuroimaging (eg, MRI) to exclude the possibility of an intracranial neoplasm.
- Compression: Localized or nonspecific headaches are common. Compression of the underlying brain can give rise to focal or more generalized cerebral dysfunction, as evinced by focal weakness, dysphasia, apathy, and/or somnolence.
- Stereotypic symptoms: Meningiomas in specific locations may give rise to the stereotyped symptoms listed in the Table. These stereotypical symptoms are not pathognomonic of meningiomas in these locations; they may occur with other conditions or lesions. Conversely, meningiomas in these locations may remain asymptomatic or produce other unlisted symptoms. Table 1. Symptoms and Signs Associated with Meningiomas in Specific Locations
Open table in new window
[ CLOSE WINDOW ]Table
Location Symptoms Parasagittal Monoparesis of the contralateral leg Subfrontal Change in mentation, apathy or disinhibited behavior, urinary incontinence Olfactory groove Anosmia with possible ipsilateral optic atrophy and contralateral papilledema (this triad termed Kennedy-Foster syndrome) Cavernous sinus Multiple cranial nerve deficits (II, III, IV, V, VI), leading to decreased vision and diplopia with associated facial numbness Occipital lobe Contralateral hemianopsia Cerebellopontine angle Decreased hearing with possible facial weakness and facial numbness Spinal cord Localized spinal pain, Brown-Sequard (hemispinal cord) syndrome Optic nerve Exophthalmos, monocular loss of vision or blindness, ipsilateral dilated pupil that does not react to direct light stimulation but might contract on consensual light stimulation; often, monocular optic nerve swelling with optociliary shunt vessels Sphenoid wing Seizures; multiple cranial nerve palsies if the superior orbital fissure involved Tentorial May protrude within supratentorial and infratentorial compartments, producing symptoms by compressing specific structures within these 2 compartments 3 Foramen magnum Paraparesis, sphincteric troubles, tongue atrophy associated with fasciculation Location Symptoms Parasagittal Monoparesis of the contralateral leg Subfrontal Change in mentation, apathy or disinhibited behavior, urinary incontinence Olfactory groove Anosmia with possible ipsilateral optic atrophy and contralateral papilledema (this triad termed Kennedy-Foster syndrome) Cavernous sinus Multiple cranial nerve deficits (II, III, IV, V, VI), leading to decreased vision and diplopia with associated facial numbness Occipital lobe Contralateral hemianopsia Cerebellopontine angle Decreased hearing with possible facial weakness and facial numbness Spinal cord Localized spinal pain, Brown-Sequard (hemispinal cord) syndrome Optic nerve Exophthalmos, monocular loss of vision or blindness, ipsilateral dilated pupil that does not react to direct light stimulation but might contract on consensual light stimulation; often, monocular optic nerve swelling with optociliary shunt vessels Sphenoid wing Seizures; multiple cranial nerve palsies if the superior orbital fissure involved Tentorial May protrude within supratentorial and infratentorial compartments, producing symptoms by compressing specific structures within these 2 compartments 3 Foramen magnum Paraparesis, sphincteric troubles, tongue atrophy associated with fasciculation - Vascular: This presentation, although rare, should be considered. Meningiomas of the skull base may narrow and even occlude important cerebral arteries, possibly presenting either as transient ischemic attack (TIA)–like episodes or as stroke.
- Miscellaneous
- Intraventricular meningiomas may present with obstructive hydrocephalus.
- Meningiomas in the vicinity of the sella turcica may produce panhypopituitarism.
- Meningiomas that compress the visual pathways produce various visual field defects, depending on their location.
- Rarely, chordoid meningiomas can present with hematologic disturbances, namely Castleman syndrome.4
Physical
The physical findings mirror the aforementioned symptoms and include signs due to raised intracranial pressure, involvement of cranial nerves, compression of the underlying parenchyma, and involvement of bone and subcutaneous tissues by the meningioma.
- Raised intracranial pressure leads to papilledema, decreased mentation and, ultimately, to brain herniation.
- Involvement of the cranial nerves may lead to anosmia, visual field defects, optic atrophy, diplopia, decreased facial sensation, facial paresis, decreased hearing, deviation of the uvula, and hemiatrophy of the tongue.
- Compression of the underlying parenchyma may give rise to pyramidal signs that are exemplified by pronator drift, hyperreflexia, positive Hoffman sign, and presence of the Babinski sign. Parietal-lobe syndrome may occur if the parietal lobes are compressed.
- Compression of the dominant (usually left) parietal lobe may give rise to Gerstmann syndrome: agraphia, acalculia, right-left disorientation, and finger agnosia.
- Compression of the nondominant (usually right) parietal lobe leads to tactile and visual extinction and neglect of the contralateral side.
- Compression of the occipital lobes leads to a congruent homonymous hemianopsia.
- Spinal meningiomas may give rise to a Brown-Sequard syndrome (ie, contralateral decreased pain sensation, ipsilateral weakness, decrease in position sense), sphincteric weakness and, ultimately, complete quadriparesis or paraparesis.
Causes
- Trauma and viruses have been investigated as possible causative agents for development of meningiomas. However, no definitive proof has yet been found.
- The role of inflammation (eg, posttraumatic insult) resulting in the upregulation of COX-2 has been investigated in the tumorogenesis of meningiomas.5
- On the other hand, the role of radiation in the genesis of meningiomas has been shown.
- Patients subjected to low-dose irradiation for tinea capitis may develop multiple meningiomas decades later in the field of irradiation.
- High-dose cranial irradiation may induce meningiomas after a short latency period.
- Genetic causes have been implicated in the development of meningiomas.
- The best-characterized and most common genetic alteration is the loss of the NF2 gene (NF2) on chromosome 22q6 . NF2 encodes a tumor suppressor known as merlin (or schwannomin).
- Of interest, the meningioma locus is close to but probably different from the gene responsible for NF2.
- Up to 60% of sporadic meningiomas were found to harbor NF2 mutations.
- Other cytogenetic alterations are chromosomal loss of 1p, 3p, 6q, and 14q.
- Loss of chromosome 10 is associated with increased tumor grade, shortened time to recurrence, and shortened survival.
- Progression to anaplastic meningioma has been associated with involvement of chromosomal site 17q.
- The following events were found to be associated with higher grades of meningiomas: loss of the tumor suppressor in lung cancer-1 gene (TSLC-1), loss of progesterone receptors, increased expression of cyclooxygenase 2 and ornithine decarboxylase.
- Monosomy of chromosome 7 is a rare cytogenetic change. However, it is frequently reported in radiation-induced meningiomas.
- The invasive potential of meningioma cells seems to be reflected by a balance between the expression of matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs).
- The most consistent chromosomal abnormality isolated in meningiomas is on the long arm of chromosome 22.
- Meningiomas can also be associated with different genetic syndromes, namely Gorlin7 and Rubinstein-Taybi syndromes8 .
- Several findings suggest an association between hormones and the risk for meningiomas, including increased incidence in women versus men and the presence of estrogen, progesterone, and androgen receptors on some of these tumors. However, the exact nature of this relationship and its implication on the management of meningiomas remain under investigation.
- Whether cell phone use increases the risk of meningiomas (and of brain tumors in general) remains of great interest, especially with the recent tremendous increase in the use of these devices worldwide. At present, the available data do not support such an association; however, all published studies have relatively small sample sizes and a short period of follow-up.9
More on Meningioma |
 Overview: Meningioma |
| Differential Diagnoses & Workup: Meningioma |
| Treatment & Medication: Meningioma |
| Follow-up: Meningioma |
| Multimedia: Meningioma |
| References |
| Further Reading |
| Next Page » |
References
Evans DG. Neurofibromatosis type 2: genetic and clinical features. Ear Nose Throat J. Feb 1999;78(2):97-100. [Medline].
Pieper DR, Al-Mefty O, Hanada Y, Buechner D. Hyperostosis associated with meningioma of the cranial base: secondary changes or tumor invasion. Neurosurgery. Apr 1999;44(4):742-6; discussion 746-7. [Medline].
Majchrzak K, Tymowski M. Surgical treatment of the tentorial and falco-tentorial junction meningiomas. Minim Invasive Neurosurg. Apr 2009;52(2):93-7. [Medline].
Arima T, Natsume A, Hatano H, Nakahara N, Fujita M, Ishii D, et al. Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg. Apr 2005;102(4):733-7. [Medline].
Ragel BT, Jensen RL, Couldwell WT. Inflammatory response and meningioma tumorigenesis and the effect of cyclooxygenase-2 inhibitors. Neurosurg Focus. 2007;23(4):E7. [Medline].
Kim JH, Lee SH, Rhee CH, et al. Loss of heterozygosity on chromosome 22q and 17p correlates with aggressiveness of meningiomas. J Neurooncol. Nov 1998;40(2):101-6. [Medline].
Albrecht S, Goodman JC, Rajagopolan S, Levy M, Cech DA, Cooley LD. Malignant meningioma in Gorlin's syndrome: cytogenetic and p53 gene analysis. Case report. J Neurosurg. Sep 1994;81(3):466-71. [Medline].
Verstegen MJ, van den Munckhof P, Troost D, Bouma GJ. Multiple meningiomas in a patient with Rubinstein-Taybi syndrome. Case report. J Neurosurg. Jan 2005;102(1):167-8. [Medline].
Milham S. Meningioma and mobile phone use. Int J Epidemiol. Apr 22 2009;[Medline].
Lee JW, Kang KW, Park SH, Lee SM, Paeng JC, Chung JK, et al. (18)F-FDG PET in the assessment of tumor grade and prediction of tumor recurrence in intracranial meningioma. Eur J Nucl Med Mol Imaging. Apr 18 2009;[Medline].
Kerim AA, Bonneville F, Jean B, Cornu P, Lejean L, Chiras J. Balloon-assisted embolization of skull base meningioma with liquid embolic agent. J Neurosurg. Apr 10 2009;[Medline].
Rosenberg LA, Prayson RA, Lee J, Reddy C, Chao ST, Barnett GH, et al. Long-term experience with World Health Organization grade III (malignant) meningiomas at a single institution. Int J Radiat Oncol Biol Phys. Jun 1 2009;74(2):427-32. [Medline].
Dutta D, Lee HN, Munshi A, Gupta T, Kane S, Sridhar E, et al. Intracerebral cystic rhabdoid meningioma. J Clin Neurosci. May 7 2009;[Medline].
Norden AD, Drappatz J, Wen PY. Advances in meningioma therapy. Curr Neurol Neurosci Rep. May 2009;9(3):231-40. [Medline].
Chamberlain MC, Tsao-Wei DD, Groshen S. Temozolomide for treatment-resistant recurrent meningioma. Neurology. Apr 13 2004;62(7):1210-2. [Medline].
Milker-Zabel S, Huber P, Schlegel W, Debus J, Zabel-du Bois A. Fractionated stereotactic radiation therapy in the management of primary optic nerve sheath meningiomas. J Neurooncol. Apr 1 2009;[Medline].
Smith JL, Vuksanovic MM, Yates BM, Bienfang DC. Radiation therapy for primary optic nerve meninigiomas. J Clin Neuroph. 1981;1:85-99. [Medline].
Mirimanoff RO. New radiotherapy technologies for meningiomas: 3D conformal radiotherapy? Radiosurgery? Stereotactic radiotherapy? Intensity-modulated radiotherapy? Proton beam radiotherapy? Spot scanning proton radiation therapy. . . or nothing at all?. Radiother Oncol. Jun 2004;71(3):247-9. [Medline].
Nutting C, Brada M, Brazil L, et al. Radiotherapy in the treatment of benign meningioma of the skull base. J Neurosurg. May 1999;90(5):823-7. [Medline].
Kondziolka D, Levy EI, Niranjan A, et al. Long-term outcomes after meningioma radiosurgery: physician and patient perspectives. J Neurosurg. Jul 1999;91(1):44-50. [Medline].
Kondziolka D, Niranjan A, Lunsford LD, Flickinger JC. Stereotactic radiosurgery for meningiomas. Neurosurg Clin N Am. Apr 1999;10(2):317-25. [Medline].
Strassner C, Buhl R, Mehdorn HM. Recurrence of intracranial meningiomas: did better methods of diagnosis and surgical treatment change the outcome in the last 30 years?. Neurol Res. Jun 2009;31(5):478-82. [Medline].
Al-Mefty O, Smith R. Clival and petroclival meningiomas. In: Al-Mefty O, ed. Meningiomas. New York, NY: Raven; 1991.
Alexiou GA, Vartholomatos G, Tsiouris S, Papadopoulos A, Kyritsis AP, Polyzoidis KS, et al. Evaluation of meningioma aggressiveness by (99m)Tc-Tetrofosmin SPECT. Clin Neurol Neurosurg. May 7 2008;[Medline].
Black P, Kathiresan S, Chung W. Meningioma surgery in the elderly: a case-control study assessing morbidity and mortality. Acta Neurochir (Wien). 1998;140(10):1013-6; discussion 1016-7. [Medline].
Cappabianca P, Cirillo S, Alfieri A, et al. Pituitary macroadenoma and diaphragma sellae meningioma: differential diagnosis on MRI. Neuroradiology. Jan 1999;41(1):22-6. [Medline].
De Monte F, Al-Mefty O. Meningiomas. In: Kaye AH, Laws ER, eds. Brain Tumors: An Encyclopedic Approach. Ediburgh, Scotland: Churchill Livingstone; 1995: 675-704.
Drummond KJ, Zhu JJ, Black PM. Meningiomas: updating basic science, management, and outcome. Neurologist. May 2004;10(3):113-30. [Medline].
Feldman RP, Marcovici A, Suarez M, Goodrich JT. Foreign body granuloma mimicking intracranial meningioma: case report and review of the literature. Neurosurgery. Apr 1999;44(4):855-8. [Medline].
Haddad GF, Al-Mefty O. Approaches to petroclival tumors. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. Vol 2. 2nd ed. New York, NY: McGraw-Hill; 1996:1695-706.
Haddad GF, Al-Mefty O. Meningiomas: an overview. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. Vol 1. 2nd ed. New York, NY: McGraw-Hill; 1996:833-42.
Haddad GF, Al-Mefty O. The road less traveled: transtemporal access to the CPA. Clinical Neurosurgery. 1994;41:150-167.
Iwai Y, Yamanaka K, Yasui T, et al. Gamma knife surgery for skull base meningiomas. The effectiveness of low-dose treatment. Surg Neurol. Jul 1999;52(1):40-4; discussion 44-5. [Medline].
Jaffrain-Rea ML, Minniti G, Santoro A, et al. Visual improvement during octreotide therapy in a case of episellar meningioma. Clin Neurol Neurosurg. Mar 1998;100(1):40-3. [Medline].
Kleihues P, Cavanee W. World Health Organization Classification of Tumours: Pathology and Genetics: Tumours of the Nervous System. Lyon, France: IARC; 2000.
Klutmann S, Bohuslavizki KH, Tietje N, et al. Clinical value of 24-hour delayed imaging in somatostatin receptor scintigraphy for meningioma. J Nucl Med. Aug 1999;40(8):1246-51. [Medline].
Kotzen RM, Swanson RM, Milhorat TH, Boockvar JA. Post-traumatic meningioma: case report and historical perspective. J Neurol Neurosurg Psychiatry. Jun 1999;66(6):796, 798. [Medline].
Larner AJ, Ball JA, Howard RS. Sarcoid tumour: continuing diagnostic problems in the MRI era. J Neurol Neurosurg Psychiatry. Apr 1999;66(4):510-2. [Medline].
Lee GK, Coel M, Ko J, Tom B. Two meningiomas detected incidentally by Tc-99m HDP bone scintigraphy during a work-up for breast cancer. Clin Nucl Med. Jul 1999;24(7):525-6. [Medline].
Liu M, Liu Y, Li X, Zhu S, Wu C. Cystic meninigioma. J Clin Neurosci. Sep 2007;14(9):856-9. [Medline].
Lusis E, Gutmann DH. Meningioma: an update. Curr Opin Neurol. Dec 2004;17(6):687-92. [Medline].
Nakasu S, Nakasu Y, Nakajima M, et al. Preoperative identification of meningiomas that are highly likely to recur. J Neurosurg. Mar 1999;90(3):455-62. [Medline].
Norden AD, Drappatz J, Wen PY. Targeted drug therapy for meningiomas. Neurosurg Focus. 2007;23(4):E12. [Medline].
Perry A, et al. Meningiomas. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. World Health Organization Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC; 164-172.
Rempel SA, Ge S, Gutierrez JA. SPARC: a potential diagnostic marker of invasive meningiomas. Clin Cancer Res. Feb 1999;5(2):237-41. [Medline].
Runzi MW, Jaspers C, Windeck R. Successful treatment of meningioma with octreotide [letter]. Lancet. May 13 1989;1(8646):1074. [Medline].
Sharif S, Brennan P, Rawluk D. Non-surgical treatment of meningioma: a case report and review. Br J Neurosurg. Aug 1998;12(4):369-72. [Medline].
Vaicys C, Schulder M, Wolansky LJ, Fromowitz FB. Falcotentorial plasmacytoma: case report. J Neurosurg. Jul 1999;91(1):132-5. [Medline].
Whittle IR, Smith C, Navoo P, Collie D. Meningiomas. Lancet. May 8 2004;363(9420):1535-43. [Medline].
Further Reading
Clinical guidelines
Improving outcomes for people with brain and other CNS tumours.
National Collaborating Centre for Cancer - National Government Agency [Non-U.S.]. 2006 Jun. 180 pages. NGC:005147
Gu ideline for the diagnosis, investigation and management of polycythaemia/erythrocytosis.
British Committee for Standards in Haematology - Professional Association. 2005 Jul. 22 pages. NGC:006179
ACR Appropriateness Criteria® orbits, vision, and visual loss.
American College of Radiology - Medical Specialty Society. 1999 (revised 2006). 9 pages. NGC:005122
Clinical trials
Phase II Trial of Sunitinib (SU011248) in Patients With Recurrent or Inoperable Meningioma
Monthly SOM230C for Recurrent or Progressive Meningioma
Biobank Meningioma: Storing Blood for Analysis of DNA and Protein of Patients With Meningioma
Related eMedicine topics
Meningioma, Brain
Meningioma, Spine
Meningioma, Optic Nerve Sheath
Meningioma, Sphenoid Wing
Skull Base Tumors
Keywords
meninges, meningeal carcinoma, meningeal cancer, arachnoidal cap cells, primary intracranial neoplasms, asymptomatic meningioma, neurofibromatosis-2, NF-2, familial meningiomas, primary intracranial tumors, hyperostosis, seizures, dysphasia, disinhibited behavior, somnolence, urinary incontinence, anosmia, ipsilateral optic atrophy
contralateral papilledema, Kennedy-Foster syndrome, diplopia, facial numbness, contralateral hemianopsia, facial weakness, Brown-Sequard syndrome, hemispinal cord syndrome, exophthalmos, monocular loss of vision, blindness, ipsilateraldilated pupil, monocular optic nerve swelling, optociliary shunt vessels, multiple cranial nerve palsies, paraparesis, sphincteric troubles, tongue atrophy
transient ischemic attack–like episodes, TIA–like episodes, stroke, intraventricular meningiomas, obstructive hydrocephalus, panhypopituitarism, visual field defects, raised intracranial pressure, brain herniation, decreased mentation, decreased facial sensation, facialparesis, decreasedhearing, deviation of uvula, hemiatrophy of tongue, pronator drift,hyperreflexia, positive Hoffman sign, Babinski sign, parietal-lobe syndrome, Gerstmann syndrome, agraphia, acalculia, right-left disorientation, finger agnosia, tactile extinction, neglect of contralateral side
visual extinction, congruent homonymous hemianopsia, spinal meningiomas, decreased pain sensation, quadriparesis, sphincteric weakness, ipsilateral weakness, decrease in position sense, cranial irradiation, chromosome 22q, merlin, schwannomin, anaplastic meningioma, monosomy of chromosome 7, loss of progesterone receptors, increased expression of ornithinedecarboxylase, increased expression of cyclooxygenase 2, radiation-induced meningiomas, matrix metalloproteinases, MMPs, tissue inhibitors of MMPs, TIMPs
