Approach

The management of meningioma requires a nuanced approach tailored to each patient’s symptoms, clinical characteristics, and tumor profile.[43] All patients should be evaluated by neurosurgeons experienced in meningioma treatment.

In general, the first decision is observation or treatment. If a patient is symptomatic or has documented growth of the tumor, then treatment is indicated. Asymptomatic, incidental lesions may be monitored by serial observation with imaging.[43]

There are no proven medical treatments for meningioma. Primary treatment options include surgical resection and radiation therapy. Choice of treatment modality is guided by symptom status and whether a patient is a suitable candidate for surgery.

Surgical resection provides pathologic diagnosis as well as therapeutic benefit. An open surgical approach is usually indicated as the goal of surgery is to resect the entire tumor along with the dural base and any involved bone.[50][59] Endoscopic transnasal approaches may be used for anterior skull base tumors or those involving the region of the sella.[60] Visual improvement has been noted to be superior in some studies of endonasal removal of tuberculum sellae meningiomas, although a higher rate of cerebrospinal fluid leak is noted.[61]

Primary treatment for meningiomas in younger patients is with surgical resection.[45] One analysis based on individual patient data found that the extent of initial surgical resection was the strongest independent prognostic factor for child and adolescent meningioma.[62]

Radiation therapy can either be with fractionated stereotactic radiation therapy (FSRT) or stereotactic radiosurgery (SRS), depending on the size of the tumor and its proximity to critical neural structures. Consensus guidelines on the delineation of target volumes for meningioma radiation therapy have been published.[63]

Asymptomatic or incidental

Asymptomatic or incidental lesions may be monitored for growth before considering treatment.[43] Conservatively managed asymptomatic meningiomas can grow in up to one third of patients over a 5-year period, although the actual growth rate has varied among studies.[64][65][66] The majority of tumors <2.5 cm have a stable course and do not show growth over a follow-up period of 5 years. The findings that correlate most consistently with tumor growth are: hyperintensity on T2-weighted imaging; larger tumor size; and younger age at presentation. Tumor calcification has been a relatively consistent finding in tumors exhibiting no growth.[65][67][68][69][70][71][72]

Guidance on monitoring frequency varies. The European Association of Neuro-Oncology (EANO) recommends annual magnetic resonance imaging (MRI) scans for suspected meningiomas for 5 years, with the interval doubling thereafter.[50] For patients with unresected meningiomas, the National Comprehensive Cancer Network (NCCN) recommends an MRI at 3, 6, and 12 months, then every 6 to 12 months for 5 years, and then every 1 to 3 years as clinically indicated.[43] In pediatric patients under observation, the Children’s Cancer and Leukemia Group advises initial surveillance imaging within 3 months for meningiomas of any grade.[45]

Serial imaging plans should be tailored to each patient's case, as some incidental meningiomas have higher risk for growth. Lesions with associated parenchymal edema should also be monitored closely if not treated. Emerging tools are available to help identify incidental meningiomas at higher risk, including the IMPACT calculator which takes into account patient age, performance status, radiologic features of the tumor, and provides a risk assessment for future growth.[73][74] IMPACT Calculator Opens in new window The IMPACT calculator can be used to inform monitoring strategies.

Good surgical candidate: symptomatic at any size

For symptomatic patients with meningiomas of any size, who are good candidates for surgery, primary treatment is with surgical resection.[43][50]

With incompletely resected tumors, or those of higher grade than World Health Organization (WHO) grade 1, adjuvant radiation therapy may be considered.[43][75] The subtotal resection plus SRS approach may be preferred in cases where surgical resection would be associated with unacceptable neurologic deficit.[76] FSRT may also be considered for adjunctive therapy, primarily in larger residual tumors or in those involving critical structures, but is less preferable in younger patients. It is generally preferred in meningiomas that may be less tolerant to a single, large dose of radiation delivered by SRS.

Radiation therapy may also be used as a primary therapy in some tumor locations, such as the cavernous sinus, where associated morbidity from cranial neuropathies is high with attempted surgical resection.[77][78][79]

Some large tumors can be considered for preoperative endovascular embolization to decrease vascularity and aid in resection of the tumor.[43] Due to increased risk of potential complications, preoperative embolization is not recommended for most meningiomas.[50] However, in select cases where there is concern that a tumor is highly vascular, it can be a useful preoperative adjunct to surgical care.

Poor surgical candidate: symptomatic and <3 cm in size

Observation is not standard of care for symptomatic tumors, but it may be considered for select patients, such as in older people.[50] Observation may continue as long as the tumor remains stable in size, although serious consideration should still be given to surgery after frank discussion with the patient and family of the risk versus benefit. Management decisions should be nuanced, based on the severity of the patient’s symptoms, tumor location, and presence of comorbidities.

If treatment is required owing to symptom progression or neurologic deterioration, primary-focused radiation (either with SRS or FSRT) is reasonable.[50][76] If the tumor progresses, surgical resection may be considered with FSRT used adjunctively, if complete surgical resection is not possible.

Poor surgical candidate: symptomatic and ≥3 cm in size

Observation is not standard of care for symptomatic tumors, but it may be considered for select patients, such as in older people.[50] Serial tumor observation with MRI may be performed if the patient has minimal symptoms (not progressing or causing significant impact on activities of daily living) and the tumor remains stable in size. Surgical resection may be necessary to reduce mass effect and critical compression of neural structures and, if required, is performed with as complete a resection as possible. It may be combined with adjuvant radiation therapy (SRS or FSRT) in cases of residual unresectable tumor.

Recurrent or progressive tumor following initial treatment

Following initial treatment for meningioma with surgical resection and/or radiation therapy, patients should be regularly monitored. Guidance on monitoring frequency varies. The EANO recommends annual MRI for 5 years for WHO grade 1 meningiomas, with the interval doubling thereafter.[50] For WHO grade 1 and 2 meningiomas, the NCCN recommends an MRI at 3, 6, and 12 months, then every 6-12 months for 5 years, and then every 1 to 3 years as clinically indicated.[43] WHO grade 3 meningiomas require more frequent monitoring; the EANO recommends at least every 3-6 months.

Treatment options for recurrent or progressive tumors are limited. Patients should be considered for referral to centers that offer clinical trials for meningioma, or who have neuro-oncology expertise in considering targeted off-label medical therapies with biologic rationale, as there are currently no approved medical therapies.[43][80]

For patients who are good surgical candidates, salvage surgery may be performed following initial failed treatment.[43] This should be combined with radiation therapy if a patient has not been initially irradiated.[50] However, re-irradiation may be considered in patients already treated with this modality. Specialized centers may also offer intracranial brachytherapy at the time of repeat surgery.[81][82]

If salvage surgery is not an option, radiation therapy alone may be considered. Treatment with systemic therapy is indicated where radiation therapy is not possible. Where treatment is not clinically indicated in any case, an observation-only approach is warranted.[43]

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