History and exam
Key diagnostic factors
common
presence of risk factors
headache
New onset headache is the most common finding among patients with meningioma (up to 37%).[49] May be progressive, focal, or general. Common in large tumours.
neurological deficit
Dependent on tumour size, location, and number. Up to 31% of patients with meningioma may present with focal cranial nerve deficit, 11% with weakness, 6% with ataxia/gait changes, and 6% with pain or other sensory changes.[49]
Other diagnostic factors
Risk factors
strong
ionizing radiation
Ionising radiation increases the risk of several intracranial tumours and, in particular, the risk of meningioma. Meningiomas that occur in patients with previous radiation exposure are often multiple and associated with high recurrence rates. Meningiomas occur at a median of 22 years after treatment with cranial irradiation for childhood cancer, with the increased risk sustained beyond age 40.[9][11] They may occur with increased frequency in patients associated with a very low dose of radiation. Children who were exposed to low-dose radiation (approximately 1.5 Gy) in Israel over 50 years ago to treat tinea capitis were observed to have a relative risk of almost 10 for meningioma.[25] Studies have also linked the number of full-mouth dental radiographs to risk of meningioma, although the sample sizes are limited and some later studies (also small in size) did not confirm these findings.[26][27]
genetic predisposition
Studies examining the relationship between a diagnosis of meningioma and a family history of meningioma are few. However, one study demonstrated a significant association between meningioma diagnosis and a parental history of meningioma.[28] Rare, but penetrant, inherited genes may exist for meningioma susceptibility, although it seems at present that these genes may be seen primarily in families with neurofibromatosis 2 (NF2).[29] In sporadic meningioma, NF2 is deleted or mutated in up to 50% of cases. In addition to NF2, chromosomes 1p, 3p, 6q, 10, and 14q are suspected locations for tumour suppressor genes.[30]
Rarer genetic syndromes associated with meningioma include familial schwannomatosis (SMARCB1), multiple spinal meningiomas (SMARCE1), BAP1 tumour predisposition syndrome, Gorlin syndrome (PTCH1/SUFU), Rubenstein-Taybi syndrome (CREBBP), and von Hippel-Lindau syndrome (VHL), among others.[20]
In addition to the familial risk associated with the presence of meningioma or in families with NF2, an association between breast cancer and meningioma has been noted in several studies.[31][32] However, one systematic review and meta-analysis did not find a statistically significant odds ratio of meningioma in female patients with breast cancer.[15] A number of explanations have been proposed for this association, including common risk factors (particularly those with a hormonal component, such as age at menopause or use of oral contraceptives) or shared genetic predisposition.
weak
hormones: endogenous and exogenous
An association between hormones and meningioma risk has been suggested by a number of findings, including the increased incidence of the disease in women versus men, an association between breast cancer and meningiomas, and indications that meningiomas change significantly in size during pregnancy.[15][33][34] The expression of oestrogen, progesterone, and androgen receptors in some meningiomas has also been reported in the literature.[35][36]
The association between exogenous hormone use and meningioma risk has been studied; however, data are sparse and inconclusive.[37][38] There is little statistical evidence of an increased risk of meningioma among users of oral contraceptives; however, one national case-control study reported an increased risk of meningioma with the use of progestins including medroxyprogesterone, medrogestone, and promegestone.[39]
An association between the use of hormone replacement therapy and increased meningioma risk has also been reported, as well as an increased incidence in women exposed to fertility treatments and in transgender people exposed to high-dose oestrogen/progesterone, although the mechanisms and causality remain to be determined.[16][19]
There is an emerging literature about the risks of cyproterone exposure and meningioma development and growth.[18][40][41] After discontinuation of cyproterone, there are reports of meningioma regression in volume.[42][43] Patients with meningiomas and exogenous hormone exposure should be referred to neurosurgeons specialised in meningioma treatment and management.
Consensus recommendations on the management of meningioma and sex hormone therapy have been published.[44]
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