Meningioma
Meningiomas grow from the membranes covering the brain and spinal cord rather than from the brain itself. Most are slow and benign and are either watched or removed; radiotherapy or radiosurgery treats what surgery cannot reach or what grows back, and no drug has yet been approved for them.
Overview
Meningiomas arise from arachnoid cap cells and are graded 1 to 3 in WHO 2021 by mitotic count, brain invasion and specific histological patterns, with two molecular criteria that assign grade 3 regardless of appearance: homozygous CDKN2A/B deletion and TERT promoter mutation. About half of sporadic tumours carry NF2 loss with monosomy 22, and most of the rest carry mutually exclusive mutations in TRAF7, KLF4, AKT1, SMO, PIK3CA or POLR2A that cluster at the skull base (Clark and Brastianos, 2013). DNA methylation classes and integrated molecular grading (Sahm 2017, Nassiri 2021) predict recurrence better than histology alone. Radiation exposure is the only established environmental cause; progesterone and oestrogen receptors explain the female excess and the link to some progestogens.
Incidental small meningiomas are watched with MRI. Symptomatic or growing tumours are resected, with completeness graded by the Simpson scale, and complete resection of a grade 1 tumour is usually curative. Radiosurgery controls most small tumours (under about 3 cm) and is the usual choice for skull base and cavernous sinus lesions that cannot be safely removed. Fractionated radiotherapy is given after incomplete resection of grade 2 tumours and after any resection of grade 3 tumours, following the phase 2 EORTC 22042-26042 and RTOG 0539 studies; whether completely resected grade 2 tumours need radiotherapy is the question of the ROAM/EORTC 1308 and NRG BN003 randomised trials. Proton therapy is used for large skull base and re-irradiation cases.
No systemic therapy is approved. Hydroxyurea, somatostatin analogues, interferon and mifepristone have all failed or shown marginal activity; bevacizumab and sunitinib produce modest control in recurrent high-grade disease, everolimus with octreotide has phase 2 activity (CEVOREM), and Alliance A071401 is testing mutation-matched drugs (the FAK inhibitor GSK2256098 in NF2-mutant tumours, SMO and AKT inhibitors, CDK inhibitors). Somatostatin receptor 2 expression makes DOTATATE PET useful for imaging and has led to trials of peptide receptor radionuclide therapy. Grade 3 and recurrent unresectable meningiomas remain a real unmet need.
State of the art
- Two molecular markers now assign grade 3 regardless of histology, and methylation classes forecast recurrence better than the microscope.
- Radiosurgery controls most small meningiomas without an operation and has become the default for skull base disease.
- Meningioma is the commonest brain tumour and still has no approved systemic therapy; mutation-matched and radionuclide trials are the first rational attempts.
Anatomy and lymph node drainage
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
- Frontal lobe (glioblastoma commonest)Meningioma, grade 3 (anaplastic; or any meningioma with CDKN2A/B homozygous deletion or TERT promoter mutation)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant gliomaMeningioma, grade 2 (atypical; chordoid and clear cell patterns) · Meningioma, grade 3 (anaplastic; or any meningioma with CDKN2A/B homozygous deletion or TERT promoter mutation)
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)Spinal meningioma (intradural extramedullary)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)Meningioma, grade 1 (meningothelial, fibrous, transitional, psammomatous and other benign patterns) · Meningioma, grade 2 (atypical; chordoid and clear cell patterns) · Meningioma, grade 3 (anaplastic; or any meningioma with CDKN2A/B homozygous deletion or TERT promoter mutation) · NF2-related meningioma (multiple, often with schwannoma) · Skull base meningioma with TRAF7, KLF4, AKT1, SMO or PIK3CA mutation (convexity tumours are mostly NF2-driven) · Spinal meningioma (intradural extramedullary)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)NF2-related meningioma (multiple, often with schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Glioma & glioblastoma
The commonest primary intracranial tumour, found in about one in a hundred adults on imaging, mostly women; the great majority are grade 1 and never threaten life, while grade 3 tumours behave like cancers and have no approved drug.
Observation with serial MRI; many never grow. Treatment when growth or symptoms appear.
Surgical resection as complete as safely possible; complete resection of a grade 1 tumour is usually curative and needs no adjuvant treatment.
Stereotactic radiosurgery (Gamma Knife, CyberKnife or linac) or fractionated stereotactic radiotherapy, with high long-term control rates for grade 1 tumours.
Fractionated radiotherapy after surgery (EORTC 22042-26042, RTOG 0539); proton therapy for large or re-irradiated skull base tumours; observation versus radiotherapy after complete resection of grade 2 tumours is under trial (ROAM/EORTC 1308, NRG BN003).
No approved drug. Bevacizumab, sunitinib or everolimus with a somatostatin analogue on phase 2 evidence; mutation-matched trials (Alliance A071401) and peptide receptor radionuclide therapy studies preferred.
Subtypes & biomarkers
top- Meningioma, grade 1 (meningothelial, fibrous, transitional, psammomatous and other benign patterns)
- Meningioma, grade 2 (atypical; chordoid and clear cell patterns)
- Meningioma, grade 3 (anaplastic; or any meningioma with CDKN2A/B homozygous deletion or TERT promoter mutation)
- NF2-related meningioma (multiple, often with schwannoma)
- Skull base meningioma with TRAF7, KLF4, AKT1, SMO or PIK3CA mutation (convexity tumours are mostly NF2-driven)
- Spinal meningioma (intradural extramedullary)
- WHO grade with mitotic count and brain invasion
- CDKN2A/B homozygous deletion and TERT promoter mutation (assign grade 3)
- NF2 loss and monosomy 22
- TRAF7, KLF4, AKT1, SMO, PIK3CA and POLR2A mutations
- DNA methylation class and integrated molecular grade
- Somatostatin receptor 2 expression (DOTATATE PET)
- Simpson grade of resection
How often this target appears
- 1922Cushing coins the term meningioma
Cushing and Eisenhardt's 1938 monograph followed, with the first large surgical series.
- 1957Simpson grades completeness of resection and links it to recurrence
- 1993NF2 gene identified on chromosome 22
Trofatter and Rouleau clone the gene whose loss drives most meningiomas and schwannomas.
- 2013Exome sequencing finds TRAF7, KLF4, AKT1 and SMO mutations in NF2-intact meningiomas
Clark and colleagues (Science) and Brastianos and colleagues (Nature Genetics).
- 2017DNA methylation classes of meningioma predict recurrence
Sahm and colleagues (Lancet Oncology).
- 2021WHO 2021 adds CDKN2A/B deletion and TERT promoter mutation as grade 3 criteria
Dated changes read from the records linked to this indication: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 7 changes by month →- 2026-09-17This recordMeningiomaFacts on this page last checked
When this page itself was last checked or edited.
- 2021MilestoneMeningiomaWHO 2021 adds CDKN2A/B deletion and TERT promoter mutation as grade 3 criteria
A milestone in how this cancer is treated.
- 2017MilestoneMeningiomaDNA methylation classes of meningioma predict recurrence
Sahm and colleagues (Lancet Oncology).
- 2013MilestoneMeningiomaExome sequencing finds TRAF7, KLF4, AKT1 and SMO mutations in NF2-intact meningiomas
Clark and colleagues (Science) and Brastianos and colleagues (Nature Genetics).
- 1993MilestoneMeningiomaNF2 gene identified on chromosome 22
Trofatter and Rouleau clone the gene whose loss drives most meningiomas and schwannomas.
- 1957MilestoneMeningiomaSimpson grades completeness of resection and links it to recurrence
A milestone in how this cancer is treated.
What is in development for Meningioma, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
No approved systemic therapy; grade 3 and recurrent unresectable tumours have few options.
Whether completely resected grade 2 meningiomas need radiotherapy (ROAM, NRG BN003).
Which incidental meningiomas will grow; most never do.
Long-term cognitive and endocrine effects of radiotherapy to the skull base.
Trials
topTrials recruiting now
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Direct links plus the targets, companies, and technologies of this cancer's products.
fronts
1technologies
1drugs
2companies
2roadmaps
2Latest papers
topQuery for this indication: (TITLE:"Meningioma" OR ABSTRACT:"Meningioma" OR TITLE:"Meningeal tumour" OR ABSTRACT:"Meningeal tumour" OR TITLE:"Atypical meningioma" OR ABSTRACT:"Atypical meningioma" OR TITLE:"Anaplastic meningioma" OR ABSTRACT:"Anaplastic meningioma" OR TITLE:"Malignant meningioma" OR ABSTRACT:"Malignant meningioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Meningioma, not a curated reading list.
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