Meningiomas: Overview and New Directions in Therapy

Nancy Wang, MD; Matthias Osswald, MD


Semin Neurol. 2018;38(1):112-120. 

In This Article

Epidemiology and Risk Factors

The incidence of meningiomas increases with age, with a median age at diagnosis of 65 years and an incidence of 7.86 per 100,000 population according to the most recent report from the Central Brain Tumor Registry of the United States.[1] The lifetime risk of developing meningioma is approximately 1%.[2] Females have a higher annual incidence than males (10.5 vs. 4.8 cases per 100,000).[1,2] Attempts to causally link endogenous or exogenous hormone exposure to meningioma have led to somewhat conflicting results, though there does appear to be an association between hormone replacement therapy and risk of meningioma.[3–9]

Exposure to ionizing radiation is the primary modifiable risk factor identified for meningioma, leading to a six- to 10-fold increase in risk.[10–12] Atomic bomb survivors in Hiroshima and Nagasaki exposed to high doses are at a significantly higher risk, as are those exposed to low doses, such as children treated with low-dose cranial radiation for tinea capitis.[13–15] Patients who receive cranial irradiation for head and neck cancers or acute lymphoblastic leukemia also have an increased risk in a dose-dependent manner.[10,16–18] Radiation-induced meningiomas are more likely to be multifocal and high grade, although a review of survivors of childhood cancer found that 5-year survival rates were similar to those with primary meningiomas.[16,17,19,20]

Meningiomas are associated with neurofibromatosis type 2 (NF2), a genetic syndrome characterized by a germline mutation on chromosome 22q12 that is inherited in an autosomal dominant pattern. Patients with NF2 tend to develop meningiomas earlier in life and are more likely to have multiple lesions, although it is unclear if these tumors behave more aggressively than sporadic cases.[21,22] Meningiomas have additionally been associated with germline mutations in SMARCB1, which also gives rise to schwannomatosis.[23] Associations with other genetic syndromes are rare, although cases have been reported in association with Gorlin's syndrome, Cowden's syndrome, and multiple endocrine neoplasia type 1.[24]