# Meningioma

Source: https://onco.cc/cancers/meningioma/  
OnCo record `meningioma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Meningeal tumour; Atypical meningioma; Anaplastic meningioma; Malignant meningioma
- Tags: subtype-page; cns
- Group: central nervous system
- Burden: 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.
- Subtypes: 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)
- Biomarkers: 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/meningioma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/meningioma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/meningioma/#what-it-is [6 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/meningioma/#finding-it [7 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/meningioma/#treating-it [5 settings, 5 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/meningioma/#evidence [2 trials, 5 key papers, 6 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/meningioma/#science [10 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/meningioma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/meningioma/#living-with-it [15 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/meningioma/coming/ [5 medicines, 2 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/meningioma/data/ [43 connected records]

## Standard of care

- Incidental or small asymptomatic: Observation with serial MRI; many never grow. Treatment when growth or symptoms appear. ([Active surveillance](https://onco.cc/technologies/active-surveillance/), [MRI](https://onco.cc/technologies/mri/))
- Symptomatic or growing, accessible: Surgical resection as complete as safely possible; complete resection of a grade 1 tumour is usually curative and needs no adjuvant treatment. ([MRI](https://onco.cc/technologies/mri/), [DNA methylation profiling](https://onco.cc/technologies/methylation-profiling/))
- Small, skull base or surgically inaccessible: Stereotactic radiosurgery (Gamma Knife, CyberKnife or linac) or fractionated stereotactic radiotherapy, with high long-term control rates for grade 1 tumours. ([Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)](https://onco.cc/technologies/radiosurgery-srs/), [Gamma Knife](https://onco.cc/technologies/gamma-knife/), [CyberKnife robotic radiosurgery](https://onco.cc/technologies/cyberknife/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Grade 2, incompletely resected, and all grade 3: 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). ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/))
- Recurrent, no surgical or radiotherapy option: 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. ([Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Sunitinib](https://onco.cc/drugs/sunitinib/), [Everolimus](https://onco.cc/drugs/everolimus/), [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/))

## 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Meningioma
- Wikipedia: https://en.wikipedia.org/wiki/Meningioma
- EANO guideline on meningiomas (Neuro-Oncology 2021): https://doi.org/10.1093/neuonc/noab150

## Connected records

- cancers: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Brain metastases (secondary brain tumours)](https://onco.cc/cancers/secondary-brain-tumours/), [Craniopharyngioma](https://onco.cc/cancers/craniopharyngioma/), [Glioma & glioblastoma](https://onco.cc/cancers/glioblastoma/), [Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma](https://onco.cc/cancers/pituitary-tumours/), [Spinal cord tumours (intramedullary and intradural)](https://onco.cc/cancers/spinal-cord-tumours/), [Vestibular schwannoma (acoustic neuroma)](https://onco.cc/cancers/vestibular-schwannoma/)
- technologies: [Active surveillance](https://onco.cc/technologies/active-surveillance/), [CyberKnife robotic radiosurgery](https://onco.cc/technologies/cyberknife/), [DNA methylation profiling](https://onco.cc/technologies/methylation-profiling/), [Gamma Knife](https://onco.cc/technologies/gamma-knife/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [MRI](https://onco.cc/technologies/mri/), [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)](https://onco.cc/technologies/radiosurgery-srs/)
- targets: [FAK (PTK2)](https://onco.cc/targets/fak/), [LEPR](https://onco.cc/targets/lepr/), [mTOR](https://onco.cc/targets/mtor/), [PTTG1](https://onco.cc/targets/pttg1/), [SMARCE1](https://onco.cc/targets/smarce1/), [SUFU](https://onco.cc/targets/sufu/)
- drugs: [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Everolimus](https://onco.cc/drugs/everolimus/), [GSK2256098](https://onco.cc/drugs/gsk2256098/), [Mifepristone](https://onco.cc/drugs/mifepristone/), [Sunitinib](https://onco.cc/drugs/sunitinib/)
- terms: [CDKN2A/B homozygous deletion](https://onco.cc/terms/cdkn2a-homozygous-deletion/), [Stereotactic radiosurgery (SRS)](https://onco.cc/terms/stereotactic-radiosurgery/)
- key papers: [Alliance A071401: phase II trial of focal adhesion kinase inhibition in meningiomas with somatic NF2 mutations](https://onco.cc/key-papers/paper-a071401-brastianos-jco-2023/), [DNA methylation-based classification and grading system for meningioma](https://onco.cc/key-papers/paper-sahm-meningioma-methylation-lancet-oncol-2017/), [EANO guideline on the diagnosis and management of meningiomas (2021)](https://onco.cc/key-papers/paper-eano-meningioma-goldbrunner-neuro-oncology-2021/), [INTUITT-NF2: brigatinib in NF2-related schwannomatosis with progressive tumours](https://onco.cc/key-papers/paper-intuitt-nf2-brigatinib-plotkin-nejm-2024/), [The 2021 WHO classification of tumours of the central nervous system: a summary](https://onco.cc/key-papers/paper-who-2021-cns-louis-neuro-oncology-2021/)
- trials: [Alliance A071401](https://onco.cc/trials/a071401/), [INTUITT-NF2](https://onco.cc/trials/intuitt-nf2/)

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JSON: https://onco.cc/api/v1/entities/meningioma.json