Meningioma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Meningioma is a relatively common neoplasm of the central nervous system that arises from arachnoidal cells which are normally involved in the protection of the brain and spinal cord by forming a thick envelope of meninges around them.[1][2][3] Meningioma was first discovered by Dr Felix Platter, a Swiss physician, in the 16th century.[4][5] Meningioma may be classified according to the histological criteria of the WHO into 3 groups: benign classic meningioma (WHO grade 1), atypical meningioma (WHO grade 2), and anaplastic malignant meningioma (WHO grade 3). Meningioma may also be classified according to the tumor location into 2 main subtypes: intradural and extradural meningioma.[1][6] There are no established direct causes for meningioma. Meningioma must be differentiated from other diseases that cause similar presentation such as schwannoma, hemangiopericytoma, and solitary fibrous tumor.[6] The incidence of meningioma is approximately 7.62 per 100,000 individuals in the United States.[7] The prevalence of meningioma was estimated to be 97.5 cases per 100,000 individuals in the United States.[8] Common risk factors in the development of meningioma are history of radiation treatment, inherited nervous system disorder, and female gender.[1][9][8] There is insufficient evidence to recommend routine screening for meningioma. If left untreated, patients with meningioma may progress to develop morning headache, focal neurological deficit, and altered mental status. [1][10] The hallmark symptom of meningioma is morning headache. Other common symptoms of meningioma include weakness, tremor, and seizures.[1][11][3] Head CT scan may be diagnostic of meningioma. Findings on CT scan suggestive of meningioma include homogeneously hyperdense lesion, calcification, hyperostosis, lytic lesions and pneumosinus dilatans.[3] Brain MRI with gadolinium is the investigation of choice for the diagnosis of meningioma. On brain MRI, meningioma is characterized by a homogeneous, well circumscribed, and extra-axial mass with a broad dural base.[3] Other imaging studies for meningioma include magnetic resonance spectroscopy, magnetic resonance perfusion, and angiography.[3] Bromodeoxyuridine labeling study may be helpful in the diagnosis of meningioma. An elevated bromodeoxyuridine labeling index is suggestive of a rapid growth rate of meningioma and a greater incidence of recurrence following surgical resection.[12] In asymptomatic meningiomas, the decision of surgical resection must be weighed against the possibility of conservative management according to the patient's age, clinical presentation, and the anatomical location of the tumor.[13][1] The predominant therapy for meningioma is surgical resection.[1] Current data suggest that both external beam radiotherapy and radiosurgery play an important role in the management of grade II and III meningiomas.[1][14]
Historical Perspective
Meningioma was first discovered by Dr Felix Platter, a Swiss physician, in the 16th century. He described the tumor as having the shape of an acorn, fleshy, and full of holes.
Classification
Meningioma may be classified according to the histological criteria of the WHO into 3 groups: benign classic meningioma (WHO grade 1) which is made up of nine variants, atypical meningioma (WHO grade 2) which is made up of three, and anaplastic malignant meningioma (WHO grade 3) made up of three variants.
Pathophysiology
Meningioma arises from the arachnoid "cap" cells, which are normally involved in the protection of the central nervous system by forming a thick envelope of meninges around the brain and spinal cord. Because of the abundance of arachnoid cap cells in the skull base and perivenous sinuses, meningiomas are commonly found in these sites. Some meningiomas may have progesterone receptors which could make the tumor grow in size during pregnancy and in the luteal phase of the menstrual cycle. Meningiomas could be found in various locations such as sphenoid ridge, olfactory grove, falx cerebri, cerebellopontine angle, foramen magnum, ventricles, etc. Though most meningiomas are benign in nature, some may be malignant and this characterization is usually based on brain invasion, frank anaplasia, and distant metastasis. NF2, MEG2, TERT, AKT1, and NDRG2 are some of the genes that may be involved in the pathogenesis of meningioma. Some conditions could be associated with meningioma, they include neurofibromatosis type 2, cowden syndrome, nevoid basal cell carcinoma, multiple endocrine neoplasia 1 (MEN1), etc. On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma. On microscopic histopathological analysis, meningiomas may have different characeteristics, they include mitotic figures, interdigitating processes and intercellular junctions, prominent nucleoli, necrosis, and increased cellularity.
Causes
There are no established direct causes for meningioma.
Differentiating Meningioma from other Diseases
Meningioma must be differentiated from other diseases that have similar presentation such as schwannoma, hemangiopericytoma, and solitary fibrous tumor.[6]
Epidemiology and Demographics
The prevalence of meningioma was estimated to be 97.5 cases per 100,000 individuals in the United States.[8] The incidence of meningioma is approximately 7.62 per 100,000 individuals in the United States.[7] Meningiomas may appear at any age, but occur most commonly among patients older than 50 years of age.[1] Females are more commonly affected with meningiomas than males.[8] Meningioma usually affects individuals of the African American race. Caucasian and Latin American individuals are less likely to develop meningioma.[8]
Risk Factors
Common risk factors in the development of meningioma are history of radiation treatment, inherited nervous system disorder, and female gender.[1][9][8]
Screening
There is insufficient evidence to recommend routine screening for meningioma.
Natural History, Complications and Prognosis
If left untreated, patients with meningioma may progress to develop morning headache, focal neurological deficit, and altered mental status. Common complications of meningioma include increased intracranial pressure, cranial nerve palsies, and hydrocephalus. Prognosis is generally good, and the survival rate of patients with meningioma mainly depends on the grade and location of the tumor.[1][10]
Diagnosis
Staging
There is no established system for the staging of meningioma.
History and Symptoms
The hallmark symptom of meningioma is morning headache. Other common symptoms of meningioma include weakness, focal neurological deficit, and confusion. However, the specific clinical presentation of meningioma is determined by the exact anatomical location of the tumor.[1][11][3]
Physical Examination
Common physical examination findings of meningioma include altered mental status, hydrocephalus, aphasia, incoordination, and sensory loss.[1][11][3]
Laboratory Findings
There are no diagnostic lab findings associated with meningioma.
X Ray
Plain radiography no longer has a role in the diagnosis or management of meningioma.[3]
CT
Head CT scan may be diagnostic of meningioma. Findings on CT scan suggestive of meningioma include homogeneously hyperdense lesion, calcification, hyperostosis, lytic lesions, and pneumosinus dilatans.[3]
MRI
Brain MRI with gadolinium is the investigation of choice for the diagnosis of meningioma. On brain MRI, meningioma is characterized by a homogeneous, well circumscribed, and extra-axial mass with a broad dural base. Other findings on brain MRI suggestive of meningioma include CSF vascular cleft sign, dural tail sign, and sunburst or spokewheel appearance of the vessels.[3]
Other Imaging Findings
Other imaging studies for meningioma include MR spectroscopy, MR perfusion, and angiography.[3] MR spectroscopy study for meningioma demonstrates elevated levels of alanine, glutamine, and choline, as well as a significantly reduced levels of N-acetylaspartate and creatine. MR perfusion study for meningioma demonstrates an elevated value of relative cerebral blood volume (rCBV).[15] Angiography of meningioma demonstrates high vascularization of the tumor due to dual blood supply by both pial (intracranial) and meningeal (extracranial) vessels.[3]
Other Diagnostic Studies
Bromodeoxyuridine labeling study may be helpful in the diagnosis of meningioma. An elevated bromodeoxyuridine labeling index is suggestive of a rapid growth rate of meningioma and a greater incidence of recurrence following surgical resection.[12]
Treatment
Medical Therapy
In asymptomatic meningiomas, the decision of surgical resection must be weighed against the possibility of conservative management according to the patient's age, clinical presentation, and the anatomical location of the tumor.[13][1] Current data suggest that both external beam radiotherapy and radiosurgery play an important role in the management of grade II and III meningiomas.[14][16] Chemotherapeutic agents are generally not effective against meningioma.[1]
Surgery
The predominant therapy for meningioma is surgical resection. Adjunctive radiation therapy may be required among certain patients.[1] The Simpson criteria for meningioma correlates the degree of surgical resection completeness with the probability of post-surgical tumor recurrence.[1][3][17] Surgical resection is not recommended among patients with asymptomatic stable meningioma.[1]
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015
- ↑ Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25 2015
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25 2015
- ↑ Felix Plater. Wikipedia(2015) https://en.wikipedia.org/wiki/Felix_Plater Accessed on September, 25 2015
- ↑ Bir SC, Maiti TK, Bollam P, Nanda A (2015). "Felix Platter and a historical perspective of the meningioma". Clin Neurol Neurosurg. 134: 75–8. doi:10.1016/j.clineuro.2015.02.018. PMID 25965286.
- ↑ 6.0 6.1 6.2 Meningioma. Liberpathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview Accessed on September, 25 2015
- ↑ 7.0 7.1 Dolecek TA, Dressler EV, Thakkar JP, Liu M, Al-Qaisi A, Villano JL (2015). "Epidemiology of meningiomas post-Public Law 107-206: The Benign Brain Tumor Cancer Registries Amendment Act". Cancer. 121 (14): 2400–10. doi:10.1002/cncr.29379. PMID 25872752.
- ↑ 8.0 8.1 8.2 8.3 8.4 8.5 Wiemels J, Wrensch M, Claus EB (2010). "Epidemiology and etiology of meningioma". J Neurooncol. 99 (3): 307–14. doi:10.1007/s11060-010-0386-3. PMC 2945461. PMID 20821343.
- ↑ 9.0 9.1 Risk factors for brain and spinal cord cancer. Canadian Cancer Society(2015) http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/risks/?region=mb Accessed on September, 25 2015
- ↑ 10.0 10.1 Fathi AR, Roelcke U (2013). "Meningioma". Curr Neurol Neurosci Rep. 13 (4): 337. doi:10.1007/s11910-013-0337-4. PMID 23463172.
- ↑ 11.0 11.1 11.2 Meningioma Brain Tumor. UCLA Neurosurgery(2015) http://neurosurgery.ucla.edu/body.cfm?id=1123&ref=62&action=detail Accessed on September, 25 2015
- ↑ 12.0 12.1 Lee KS, Hoshino T, Rodriguez LA, Bederson J, Davis RL, Wilson CB (1990). "Bromodeoxyuridine labeling study of intracranial meningiomas: proliferative potential and recurrence". Acta Neuropathol. 80 (3): 311–7. PMID 2399811.
- ↑ 13.0 13.1 Herscovici Z, Rappaport Z, Sulkes J, Danaila L, Rubin G (2004). "Natural history of conservatively treated meningiomas". Neurology. 63 (6): 1133–4. PMID 15452322.
- ↑ 14.0 14.1 Maclean J, Fersht N, Short S (2014). "Controversies in radiotherapy for meningioma". Clin Oncol (R Coll Radiol). 26 (1): 51–64. doi:10.1016/j.clon.2013.10.001. PMID 24207113.
- ↑ Zimny A, Sasiadek M (2011). "Contribution of perfusion-weighted magnetic resonance imaging in the differentiation of meningiomas and other extra-axial tumors: case reports and literature review". J Neurooncol. 103 (3): 777–83. doi:10.1007/s11060-010-0445-9. PMC 3116130. PMID 21061142.
- ↑ Ding D, Starke RM, Hantzmon J, Yen CP, Williams BJ, Sheehan JP (2013). "The role of radiosurgery in the management of WHO Grade II and III intracranial meningiomas". Neurosurg Focus. 35 (6): E16. doi:10.3171/2013.9.FOCUS13364. PMID 24289124.
- ↑ Simpson Grading System. Neurosurgic.com(2015) http://www.neurosurgic.com/index.php?option=com_content&view=article&id=846:simpson-grading-system-for-removal-of-meningeomas&catid=152:usefulinfo&Itemid=603 Accessed on September, 25 2015