Meningioma

Neoplastic MONDO:0016642 Pathograph 25 Central nervous system neoplasm meningeal neoplasm

Meningioma is a primarily meningeal central nervous system tumor with a broad clinical spectrum from lower-grade tumors to aggressive high-grade disease.

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11
Pathophys.
2
Histopath.
4
Phenotypes
25
Pathograph
13
Genes
5
Medical Actions
3
Subtypes
1
Differentials
2
Datasets
32
References
1
Deep Research
◆

Subtypes

3
Benign meningioma (WHO grade I) MONDO:0003054
Lower-grade meningioma subtype with the most favorable clinical course and the highest post-treatment progression-free survival among WHO grade groups.
Show evidence (1 reference)
PMID:40940847 SUPPORT Human Clinical
"For WHO Grade I tumors, 3- to 5-year progression-free survival (PFS) ranged from 85% to 100%."
This recurrent-meningioma SRS review supports favorable grade I progression-free survival compared with higher grades.
Atypical meningioma (WHO grade II) MONDO:0045056
Intermediate-grade meningioma subtype with elevated recurrence risk compared with grade I tumors.
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
The cohort study directly frames grade II tumors as aggressive and recurrence-prone.
Anaplastic meningioma (WHO grade III) MONDO:0020635
High-grade meningioma subtype associated with aggressive growth and treatment resistance.
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
The study identifies anaplastic meningioma as part of the high-grade aggressive spectrum.
⚙

Pathophysiology

11
NF2/Merlin Loss
NF2 loss inactivates the Merlin tumor suppressor, one of the central early genetic events in meningioma tumorigenesis and a major axis separating NF2 altered from non-NF2 molecular groups.
NF2 hgnc:7773
Downstream
Hippo-YAP Signaling Deregulation Merlin loss releases Hippo pathway control of YAP1.
Show evidence (2 references)
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT Human Clinical
"Loss of the NF2 tumor suppressor gene is the most common genetic alteration in meningiomas, and the NF2 gene product, Merlin, acts upstream of the Hippo pathway."
This establishes NF2/Merlin loss as a central meningioma driver and links it to Hippo pathway control.
DOI:10.3390/cancers15112945 SUPPORT Human Clinical
"Meningiomas in the second group have an intermediate prognosis and are characterized by NF2 alterations, mild chromosomal instability, and enrichment in immune cells."
Molecular profiling review supports NF2-altered meningioma groups as clinically meaningful disease biology.
Hippo-YAP Signaling Deregulation
Merlin loss disrupts Hippo pathway control and permits YAP1 activation, promoting meningioma cell proliferation, migration, and tumorigenic behavior.
NF2 hgnc:7773 YAP1 hgnc:16262
Downstream
Cell Proliferation and Migration YAP1 activation promotes proliferative and migratory phenotypes in meningioma cells.
Show evidence (2 references)
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT Human Clinical
"Here, we show that primary meningioma tumors have high nuclear expression of YAP1."
Primary tumor data support YAP1 activation in human meningioma.
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT In Vitro
"Together, these findings suggest that in meningiomas, deregulation of the Hippo pathway is largely observed in primary tumors and that YAP1 functions as an oncogene promoting meningioma tumorigenesis."
The study directly connects Hippo pathway deregulation and oncogenic YAP1 function in meningioma.
Cell Proliferation and Migration
YAP1 activation promotes meningioma-cell proliferation, migration, and anchorage-independent growth downstream of Hippo pathway deregulation.
cell population proliferation GO:0008283 ↑ INCREASED cell migration GO:0016477 ↑ INCREASED
Show evidence (2 references)
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT In Vitro
"Using an siRNA transient knockdown of YAP1 in NF2-mutant meningioma cells, we show that suppression of YAP1 impaired cell proliferation and migration."
YAP1 knockdown reducing proliferation and migration supports the downstream cellular phenotype.
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT In Vitro
"Conversely, YAP1 overexpression led to a strong augment of cell proliferation and anchorage-independent growth and restriction of cisplatin-induced apoptosis."
YAP1 overexpression increasing proliferation and anchorage-independent growth supports the same downstream mechanism.
Non-NF2 Driver Mutation Subgroups
A substantial subset of meningiomas lacks NF2 alteration and instead carries mutually exclusive driver mutations in TRAF7, KLF4, AKT1, SMO, and related signaling genes, producing clinically and anatomically distinct molecular subgroups.
TRAF7 hgnc:20456 KLF4 hgnc:6348 AKT1 hgnc:391 SMO hgnc:11119 PIK3CA hgnc:8975 POLR2A hgnc:9187
Show evidence (4 references)
PMID:23348505 SUPPORT Human Clinical
"Besides NF2, we identified increased mutation burden in TNF receptor-associated factor 7 (TRAF7), Krupple-like factor 4 (KLF4), v-akt murine thymoma viral oncogene homolog 1 (AKT1), and Smoothened, frizzled family receptor (SMO) (as a group, referred to as non-NF2 mutant hereafter) (Fig. 1)."
Genomic sequencing supports TRAF7, KLF4, AKT1, and SMO as recurrent non-NF2 meningioma drivers.
PMID:23348505 SUPPORT Human Clinical
"Mutations in these genes were mutually exclusive of NF2 mutations."
This supports distinct non-NF2 driver subgroups rather than random co-occurring variants.
PMID:23334667 SUPPORT Human Clinical
"A subset of meningiomas lacking NF2 alterations harbored recurrent oncogenic mutations in AKT1 (p.Glu17Lys) and SMO (p.Trp535Leu) and exhibited immunohistochemical evidence of activation of these pathways."
Independent genomic sequencing supports AKT1 and SMO mutations in the non-NF2 meningioma subset.
+ 1 more reference
CDKN2A/B Deletion-Associated Progression
CDKN2A and CDKN2B deletion marks aggressive meningioma biology and is linked to shorter progression-free survival, with homozygous deletion incorporated into modern high-grade risk assessment.
CDKN2A hgnc:1787 CDKN2B hgnc:1788
Show evidence (2 references)
DOI:10.1186/s40478-023-01690-y SUPPORT Human Clinical
"Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression."
Patient-level meta-analysis supports CDKN2A/B deletion as progression-associated meningioma biology.
DOI:10.3390/cancers15112945 SUPPORT Human Clinical
"The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk."
WHO-linked molecular criteria support CDKN2A/B homozygous deletion as a high-risk meningioma feature.
TERT Promoter Mutation-Associated Grade 3 Biology
TERT promoter mutations identify a molecularly high-risk meningioma subset associated with recurrence risk and WHO grade 3 assignment in modern classification.
TERT hgnc:11730
Show evidence (1 reference)
DOI:10.3390/cancers15112945 SUPPORT Human Clinical
"The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk."
This directly supports TERT promoter mutation status as a grade 3, recurrence-associated molecular feature.
NOTCH3 Activation in Aggressive Meningioma
NOTCH3 signaling is associated with aggressive meningioma behavior and links to downstream metabolic reprogramming.
NOTCH3 hgnc:7883
Downstream
CD36-Associated Fatty Acid Oxidation Reprogramming NOTCH3-high states correlate with FAO-related metabolic features.
Show evidence (2 references)
PMID:40924320 SUPPORT In Vitro
"While prior work has linked NOTCH3 expression to higher-grade meningiomas and treatment resistance, the metabolic phenotype of NOTCH3 activation remains unexplored in meningioma."
Supports NOTCH3 as a marker of aggressive, treatment-resistant meningioma states.
PMID:40924320 SUPPORT In Vitro
"We show that NOTCH3 mediates a metabolic shift towards fatty acid oxidation (FAO), depleting lipid availability and conferring resistance to ferroptosis."
Directly links NOTCH3 activation to metabolic rewiring in aggressive meningioma models.
CD36-Associated Fatty Acid Oxidation Reprogramming
NOTCH3-associated meningioma states show increased CD36-linked fatty acid utilization and FAO-like mitochondrial metabolism.
CD36 hgnc:1663
fatty acid transport GO:0015908 ↑ INCREASED fatty acid beta-oxidation GO:0006635 ↑ INCREASED
Downstream
Ferroptosis Resistance Enhanced FAO-linked metabolism is associated with ferroptosis evasion.
Show evidence (2 references)
PMID:40924320 SUPPORT In Vitro
"Single-cell RNA sequencing revealed a correlation with CD36, a key fatty acid transporter."
Supports CD36-linked fatty acid substrate routing in NOTCH3-associated states.
PMID:40924320 SUPPORT In Vitro
"NOTCH3 ICD overexpression (OE) exhibited depletion of fatty acid pools, alongside transcriptional upregulation of canonical FAO genes."
Supports enhanced FAO transcriptional programming under NOTCH3 activation.
Ferroptosis Resistance
Aggressive meningioma cells with activated NOTCH3 signaling display reduced sensitivity to ferroptosis, indicating a potential mechanism of treatment resistance.
ferroptosis GO:0097707 ↓ DECREASED
Show evidence (1 reference)
PMID:40924320 SUPPORT In Vitro
"Additionally, NOTCH3 OE cells exhibit increased resistance to RSL3-induced ferroptosis, a phenotype that was reversed with CPT1 inhibition."
The abstract reports ferroptosis resistance and partial reversibility with metabolic inhibition.
NY-ESO-1-Associated Immune Vulnerability in Malignant Meningioma
Higher NY-ESO-1 expression in malignant meningioma is linked to higher tumor grade and may expose a targetable immune vulnerability.
CTAG1B hgnc:2491
Downstream
NY-ESO-1-Dependent TCR-T Cytolysis Susceptibility Higher NY-ESO-1 expression increases susceptibility to NY-ESO-1-directed TCR-T killing.
Show evidence (1 reference)
PMID:40802116 SUPPORT Human Clinical
"NY-ESO-1 expression correlated with tumor grade (n = 35; p < 0.01)."
Human tumor specimens show NY-ESO-1 tracks with higher-grade disease.
NY-ESO-1-Dependent TCR-T Cytolysis Susceptibility
Meningioma cells with higher NY-ESO-1 expression show stronger cytolytic response to NY-ESO-1-targeted TCR-transduced T cells.
Show evidence (2 references)
PMID:40802116 SUPPORT In Vitro
"CH157-HLA-A2.1 cells, with native high NY-ESO-1 expression, experienced > 60% and then nearly 100% cytolysis after co-culture with TCR-T for 10 and 24 h, respectively, compared with control T-cells (p < 0.0001)."
Demonstrates expression-linked cytolytic sensitivity to NY-ESO-1-targeted TCR-T.
PMID:40802116 SUPPORT Model Organism
"Systemic ACT results in significantly increased survival in vivo in high-grade meningioma."
In vivo model data support therapeutic translation of NY-ESO-1-directed ACT.
✶

Histopathology

2
Histopathologically Confirmed High-Grade Meningioma
Recurrent/residual high-grade meningioma cohorts treated with SRS include histopathologically confirmed atypical or anaplastic tumors.
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"All patients had undergone craniotomy and histopathologic confirmation of atypical or anaplastic meningioma."
Confirms grade II/III pathology as a defining tissue-level feature in the studied cohort.
Predominance of Atypical Histology in High-Grade Cohort
In a large recurrent/residual high-grade series, atypical histology (grade II) was substantially more frequent than anaplastic histology (grade III).
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"Atypical meningioma accounted for the vast majority of cases (105; 93.8%) with only 7 cases of anaplastic meningioma (6.2%)."
Quantifies the high-grade histology distribution in a treated clinical cohort.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Meningioma Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

4
Nervous System 2
Seizure at diagnosis OCCASIONAL Seizure HP:0001250
Show evidence (1 reference)
PMID:40645150 SUPPORT Human Clinical
"new-onset seizures were reported by 26% of newly diagnosed meningioma patients."
Meta-analysis quantifies seizure presentation at diagnosis.
Headache Headache HP:0002315
Show evidence (1 reference)
PMID:40178655 SUPPORT Human Clinical
"Common and non-specific symptoms such as headache and dizziness may be wrongfully attributed to meningiomas, which can lead to unnecessary surgery and anxiety for the patient."
Supports headache as a frequent, non-specific symptom context in incidental meningioma evaluation.
Other 2
High recurrence risk in high-grade disease
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
The large retrospective cohort highlights recurrence as a defining high-grade phenotype.
Shorter progression-free survival with high NY-ESO-1 expression
Show evidence (1 reference)
PMID:40802116 SUPPORT Human Clinical
"High NY-ESO-1 nuclear expression predicted a worse progression-free-survival (p = 0.0167)."
The abstract provides a direct prognostic association between NY-ESO-1 and worse PFS.
🧬

Genetic Associations

13
NF2 (Recurrent tumor suppressor loss)
Gene: NF2 hgnc:7773
Show evidence (1 reference)
DOI:10.1158/1541-7786.MCR-12-0116 SUPPORT Human Clinical
"Loss of the NF2 tumor suppressor gene is the most common genetic alteration in meningiomas, and the NF2 gene product, Merlin, acts upstream of the Hippo pathway."
Establishes NF2 loss as a common meningioma genetic alteration.
CDKN2A (Homozygous or heterozygous deletion associated with progression)
Gene: CDKN2A hgnc:1787
Show evidence (1 reference)
DOI:10.1186/s40478-023-01690-y SUPPORT Human Clinical
"Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression."
Supports CDKN2A deletion as part of the progression-associated CDKN2A/B locus.
CDKN2B (Homozygous or heterozygous deletion associated with progression)
Gene: CDKN2B hgnc:1788
Show evidence (1 reference)
DOI:10.1186/s40478-023-01690-y SUPPORT Human Clinical
"Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression."
Supports CDKN2B deletion as part of the progression-associated CDKN2A/B locus.
TERT (Promoter mutation associated with WHO grade 3 biology)
Gene: TERT hgnc:11730
Show evidence (1 reference)
DOI:10.3390/cancers15112945 SUPPORT Human Clinical
"The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk."
Supports TERT promoter mutation as a high-grade molecular criterion.
TRAF7 (Recurrent non-NF2 driver mutation)
Gene: TRAF7 hgnc:20456
Show evidence (1 reference)
PMID:23348505 SUPPORT Human Clinical
"Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
Supports TRAF7 as part of the recurrent non-NF2 driver mutation landscape.
KLF4 (Recurrent non-NF2 driver mutation)
Gene: KLF4 hgnc:6348
Show evidence (1 reference)
PMID:23348505 SUPPORT Human Clinical
"Mutations in TRAF7 commonly occurred with a recurrent mutation (K409Q) in KLF4, a transcription factor known for its role in inducing pluripotency, or with AKT1(E17K), a mutation known to activate the PI3K pathway."
Supports recurrent KLF4 mutation as part of the non-NF2 meningioma driver landscape.
AKT1 (Recurrent activating mutation in non-NF2 meningioma)
Gene: AKT1 hgnc:391
Show evidence (1 reference)
PMID:23348505 SUPPORT Human Clinical
"The known neoplasia-related recurrent mutation, AKT1E17K, was identified in 38 meningiomas."
Supports AKT1 E17K as a recurrent meningioma driver mutation.
SMO (Recurrent Hedgehog-pathway driver mutation)
Gene: SMO hgnc:11119
Show evidence (1 reference)
PMID:23348505 SUPPORT Human Clinical
"Finally, in 11 tumors, we identified mutations in SMO, which is expressed in meningiomas (fig. S5)."
Supports SMO as a recurrent non-NF2 meningioma driver.
PIK3CA (Less common PI3K-pathway alteration)
Gene: PIK3CA hgnc:8975
Show evidence (1 reference)
PMID:23348505 SUPPORT Human Clinical
"In addition, we identified single mutations in genes previously reported to play a role in other neoplasias, including CREBBP, PIK3CA (R108H variant), PIK3R1 (deletion p.306-307), and BRCA1 as well as two SMARCB1 mutations, which coexisted with NF2 loss and have previously been reported in..."
Supports PIK3CA as a less common but reported PI3K-pathway alteration in meningioma sequencing.
POLR2A (Recurrent somatic mutation defining a distinct non-NF2 subset)
Gene: POLR2A hgnc:9187
Show evidence (1 reference)
DOI:10.1038/ng.3651 SUPPORT Human Clinical
"Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas"
Title-level cached reference supports POLR2A as a recurrent meningioma mutation subgroup.
NOTCH3 (Upregulated signaling in aggressive meningioma models)
Gene: NOTCH3 hgnc:7883
Show evidence (1 reference)
PMID:40924320 SUPPORT In Vitro
"While prior work has linked NOTCH3 expression to higher-grade meningiomas and treatment resistance, the metabolic phenotype of NOTCH3 activation remains unexplored in meningioma."
Supports NOTCH3 as an aggressive-disease-associated molecular feature.
CD36 (Correlated with NOTCH3-high metabolic state)
Gene: CD36 hgnc:1663
Show evidence (1 reference)
PMID:40924320 SUPPORT In Vitro
"Single-cell RNA sequencing revealed a correlation with CD36, a key fatty acid transporter."
Supports CD36 as a linked metabolic mediator in aggressive meningioma cells.
CTAG1B (NY-ESO-1) (Higher expression in higher-grade malignant meningioma)
Gene: CTAG1B hgnc:2491
Show evidence (1 reference)
PMID:40802116 SUPPORT Human Clinical
"NY-ESO-1 expression correlated with tumor grade (n = 35; p < 0.01)."
Supports grade-associated CTAG1B/NY-ESO-1 biology in malignant meningioma.
💊

Medical Actions

5
Gross total surgical resection
Action: surgical procedure MAXO:0000004
Surgical intervention with tissue diagnosis remains central to definitive classification; gross total resection is frequently curative when feasible.
Show evidence (1 reference)
PMID:34181733 SUPPORT Human Clinical
"A gross total surgical resection including the involved dura is often curative."
EANO guideline supports gross total resection as a key curative-intent approach.
Watch-and-scan surveillance for selected incidental tumors
Action: supportive care MAXO:0000950
Asymptomatic or elderly patients with incidentally diagnosed meningioma may be managed conservatively with serial imaging surveillance.
Show evidence (1 reference)
PMID:34181733 SUPPORT Human Clinical
"a significant proportion of meningiomas, notably in patients that are asymptomatic or elderly or both, may be managed by a watch-and-scan strategy."
Guideline-endorsed conservative management option for selected patients.
Repeated stereotactic radiosurgery (SRS)
Action: stereotactic radiosurgery MAXO:0009088
Repeated SRS is used for recurrent or residual high-grade meningioma and has a manageable toxicity profile in retrospective clinical experience.
Show evidence (1 reference)
PMID:40802120 SUPPORT Human Clinical
"Our data suggest that SRS is a relatively safe and effective treatment option for recurrent or residual high-grade meningioma, with an acceptable complication profile, even when performed repeatedly."
Human cohort data support repeated radiosurgery as a practical salvage option.
Fractionated radiotherapy for inoperable or recurrent disease
Action: radiation therapy MAXO:0000014
Fractionated RT is an accepted treatment option when tumors are inoperable or recur and require additional local control.
Show evidence (1 reference)
PMID:34181733 SUPPORT Human Clinical
"Inoperable or recurrent tumors requiring treatment can be treated with radiosurgery, if the size or the vicinity of critical structures allows that, or with fractionated radiotherapy (RT)."
EANO guidance supports fractionated RT as a standard local modality in selected cases.
NY-ESO-1 TCR-transduced T-cell therapy (investigational)
Action: cellular therapy MAXO:0000016
Preclinical adoptive cell transfer using NY-ESO-1-directed TCR-T cells shows anti-tumor activity and survival benefit in high-grade meningioma models.
Mechanism Target:
INHIBITS NY-ESO-1-Associated Immune Vulnerability in Malignant Meningioma — NY-ESO-1-targeted TCR-T cells reduce viability of NY-ESO-1-high malignant meningioma cells.
Show evidence (1 reference)
PMID:40802116 SUPPORT In Vitro
"CH157-HLA-A2.1 cells, with native high NY-ESO-1 expression, experienced > 60% and then nearly 100% cytolysis after co-culture with TCR-T for 10 and 24 h, respectively, compared with control T-cells (p < 0.0001)."
Supports mechanism-linked killing of NY-ESO-1-high malignant meningioma cells by TCR-T.
Show evidence (1 reference)
PMID:40802116 SUPPORT Model Organism
"Systemic ACT results in significantly increased survival in vivo in high-grade meningioma."
In vivo preclinical data support NY-ESO-1-directed ACT as a candidate strategy.
🔬

Biochemical Markers

1
High NY-ESO-1 Nuclear Expression (Increased in higher-grade malignant meningioma)
Show evidence (1 reference)
PMID:40802116 SUPPORT Human Clinical
"High NY-ESO-1 nuclear expression predicted a worse progression-free-survival (p = 0.0167)."
Supports NY-ESO-1 expression as a prognostic molecular feature.
🔀

Differential Diagnoses

1

Conditions with similar clinical presentations that must be differentiated from Meningioma:

Intracranial solitary fibrous tumor MONDO:0016238
Overlapping Features Intracranial SFT can mimic meningioma on imaging, making preoperative differentiation difficult.
Distinguishing Features
  • Multiparametric MRI-based segmentation and fusion models can improve preoperative discrimination between SFT and meningioma.
Show evidence (1 reference)
PMID:40625299 SUPPORT Human Clinical
"Their similar imaging features make preoperative differentiation difficult, resulting in high misdiagnosis rates."
Directly supports SFT as a clinically relevant differential diagnosis.
📊

Related Datasets

2
Development, validation, and clinical utility of a novel methylation classifier for recurrence risk prediction in meningiomas geo:GSE292327
DNA methylation profiling cohort used to develop and validate recurrence risk prediction models for meningioma.
human METHYLATION n=223
meningioma tissue
Conditions: meningioma recurrence risk stratification
PMID:41466325
GEO metadata indicates a clinically validated recurrence-risk methylation classifier cohort.
Show evidence (1 reference)
PMID:41466325 SUPPORT Human Clinical
"Using samples from 217 patients, we developed, validated, and implemented a clinically applicable methylation classifier for prognostic stratification of meningiomas based on k-means clustering of methylation data."
Supports the recurrence-risk methylation stratification intent of this dataset entry.
Single-nuclei RNA-seq of meningioma geo:GSE313693
Single-nuclei transcriptomic dataset used to characterize meningioma microenvironmental states and refine subtype/risk continuum analyses.
human SINGLE CELL RNA SEQ n=26
meningioma tissue nuclei
Conditions: meningioma microenvironment profiling
PMID:41663806
GEO metadata reports single-nuclei RNA sequencing for meningioma subtype refinement.
Show evidence (1 reference)
PMID:41663806 SUPPORT Human Clinical
"Here, by applying multiomic profiling and multiple lines of orthogonal computational evaluation in multiple independent datasets, we found that not only tumor cell characteristics but also incremental changes in the tumor microenvironment (TME) have impact on epigenetic meningioma classification..."
Supports multiomic dataset use for microenvironment-linked meningioma classification refinement.
{ }

Source YAML

click to show 
name: Meningioma
creation_date: '2026-03-03T21:32:57Z'
updated_date: '2026-05-09T19:42:12Z'
description: >-
  Meningioma is a primarily meningeal central nervous system tumor with a broad
  clinical spectrum from lower-grade tumors to aggressive high-grade disease.
category: Neoplastic
categories:
- Central Nervous System Tumor
- Meningeal Tumor
parents:
- Central nervous system neoplasm
- meningeal neoplasm
disease_term:
  preferred_term: meningioma
  term:
    id: MONDO:0016642
    label: meningioma
has_subtypes:
- name: WHO Grade I
  display_name: Benign meningioma (WHO grade I)
  description: >-
    Lower-grade meningioma subtype with the most favorable clinical course and
    the highest post-treatment progression-free survival among WHO grade groups.
  subtype_term:
    preferred_term: benign meningioma
    term:
      id: MONDO:0003054
      label: benign meningioma
  evidence:
  - reference: PMID:40940847
    reference_title: "Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "For WHO Grade I tumors, 3- to 5-year progression-free survival (PFS) ranged from 85% to 100%."
    explanation: This recurrent-meningioma SRS review supports favorable grade I progression-free survival compared with higher grades.
- name: WHO Grade II
  display_name: Atypical meningioma (WHO grade II)
  description: >-
    Intermediate-grade meningioma subtype with elevated recurrence risk
    compared with grade I tumors.
  subtype_term:
    preferred_term: grade II meningioma
    term:
      id: MONDO:0045056
      label: grade II meningioma
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
    explanation: The cohort study directly frames grade II tumors as aggressive and recurrence-prone.
- name: WHO Grade III
  display_name: Anaplastic meningioma (WHO grade III)
  description: >-
    High-grade meningioma subtype associated with aggressive growth and treatment
    resistance.
  subtype_term:
    preferred_term: anaplastic meningioma
    term:
      id: MONDO:0020635
      label: anaplastic meningioma
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
    explanation: The study identifies anaplastic meningioma as part of the high-grade aggressive spectrum.
prevalence:
- population: Individuals with incidental brain MRI findings in a population-based 70-year cohort
  percentage: 1.8
  notes: Observed prevalence in an age-homogeneous population cohort.
  evidence:
  - reference: PMID:40178655
    reference_title: "Prevalence and symptoms of incidental meningiomas: a population-based study."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The prevalence of meningioma was 1.8% (n = 14)."
    explanation: Provides a direct population-based prevalence estimate in older adults.
- population: Proportion among central nervous system neoplasms
  percentage: 40
  notes: Relative burden among CNS neoplasms in a large meta-analytic literature summary.
  evidence:
  - reference: PMID:40645150
    reference_title: "Predicting epilepsy in patients diagnosed with intracranial meningiomas: A systematic review and meta-analysis of clinical and anatomical risk factors."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Meningiomas comprise 40% of all central nervous system neoplasms, with peak prevalence occurring in the fifth and sixth decades of life."
    explanation: Supports meningioma as a major share of CNS neoplasms and age-related prevalence peak.
pathophysiology:
- name: NF2/Merlin Loss
  description: >-
    NF2 loss inactivates the Merlin tumor suppressor, one of the central early
    genetic events in meningioma tumorigenesis and a major axis separating NF2
    altered from non-NF2 molecular groups.
  genes:
  - preferred_term: NF2
    term:
      id: hgnc:7773
      label: NF2
  downstream:
  - target: Hippo-YAP Signaling Deregulation
    description: Merlin loss releases Hippo pathway control of YAP1.
    evidence:
    - reference: DOI:10.1158/1541-7786.MCR-12-0116
      reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Loss of the NF2 tumor suppressor gene is the most common genetic alteration in meningiomas, and the NF2 gene product, Merlin, acts upstream of the Hippo pathway.
      explanation: This directly supports the NF2/Merlin to Hippo pathway edge in meningioma biology.
  evidence:
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Loss of the NF2 tumor suppressor gene is the most common genetic alteration in meningiomas, and the NF2 gene product, Merlin, acts upstream of the Hippo pathway.
    explanation: This establishes NF2/Merlin loss as a central meningioma driver and links it to Hippo pathway control.
  - reference: DOI:10.3390/cancers15112945
    reference_title: "Epigenetic, Genetic, and Transcriptomic Profiling of Meningiomas: Molecular Patterns Correlate with Clinical Prognosis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Meningiomas in the second group have an intermediate prognosis and are characterized by NF2 alterations, mild chromosomal instability, and enrichment in immune cells.
    explanation: Molecular profiling review supports NF2-altered meningioma groups as clinically meaningful disease biology.
- name: Hippo-YAP Signaling Deregulation
  description: >-
    Merlin loss disrupts Hippo pathway control and permits YAP1 activation,
    promoting meningioma cell proliferation, migration, and tumorigenic behavior.
  genes:
  - preferred_term: NF2
    term:
      id: hgnc:7773
      label: NF2
  - preferred_term: YAP1
    term:
      id: hgnc:16262
      label: YAP1
  downstream:
  - target: Cell Proliferation and Migration
    description: YAP1 activation promotes proliferative and migratory phenotypes in meningioma cells.
    evidence:
    - reference: DOI:10.1158/1541-7786.MCR-12-0116
      reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: Using an siRNA transient knockdown of YAP1 in NF2-mutant meningioma cells, we show that suppression of YAP1 impaired cell proliferation and migration.
      explanation: Functional knockdown links YAP1 activity to meningioma proliferation and migration.
  evidence:
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Here, we show that primary meningioma tumors have high nuclear expression of YAP1.
    explanation: Primary tumor data support YAP1 activation in human meningioma.
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: Together, these findings suggest that in meningiomas, deregulation of the Hippo pathway is largely observed in primary tumors and that YAP1 functions as an oncogene promoting meningioma tumorigenesis.
    explanation: The study directly connects Hippo pathway deregulation and oncogenic YAP1 function in meningioma.
- name: Cell Proliferation and Migration
  description: >-
    YAP1 activation promotes meningioma-cell proliferation, migration, and
    anchorage-independent growth downstream of Hippo pathway deregulation.
  biological_processes:
  - preferred_term: cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008283
      label: cell population proliferation
  - preferred_term: cell migration
    modifier: INCREASED
    term:
      id: GO:0016477
      label: cell migration
  evidence:
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: Using an siRNA transient knockdown of YAP1 in NF2-mutant meningioma cells, we show that suppression of YAP1 impaired cell proliferation and migration.
    explanation: YAP1 knockdown reducing proliferation and migration supports the downstream cellular phenotype.
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: Conversely, YAP1 overexpression led to a strong augment of cell proliferation and anchorage-independent growth and restriction of cisplatin-induced apoptosis.
    explanation: YAP1 overexpression increasing proliferation and anchorage-independent growth supports the same downstream mechanism.
- name: Non-NF2 Driver Mutation Subgroups
  description: >-
    A substantial subset of meningiomas lacks NF2 alteration and instead carries
    mutually exclusive driver mutations in TRAF7, KLF4, AKT1, SMO, and related
    signaling genes, producing clinically and anatomically distinct molecular
    subgroups.
  genes:
  - preferred_term: TRAF7
    term:
      id: hgnc:20456
      label: TRAF7
  - preferred_term: KLF4
    term:
      id: hgnc:6348
      label: KLF4
  - preferred_term: AKT1
    term:
      id: hgnc:391
      label: AKT1
  - preferred_term: SMO
    term:
      id: hgnc:11119
      label: SMO
  - preferred_term: PIK3CA
    term:
      id: hgnc:8975
      label: PIK3CA
  - preferred_term: POLR2A
    term:
      id: hgnc:9187
      label: POLR2A
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Besides NF2, we identified increased mutation burden in TNF receptor-associated factor 7 (TRAF7), Krupple-like factor 4 (KLF4), v-akt murine thymoma viral oncogene homolog 1 (AKT1), and Smoothened, frizzled family receptor (SMO) (as a group, referred to as non-NF2 mutant hereafter) (Fig. 1).
    explanation: Genomic sequencing supports TRAF7, KLF4, AKT1, and SMO as recurrent non-NF2 meningioma drivers.
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Mutations in these genes were mutually exclusive of NF2 mutations.
    explanation: This supports distinct non-NF2 driver subgroups rather than random co-occurring variants.
  - reference: PMID:23334667
    reference_title: Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations.
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: A subset of meningiomas lacking NF2 alterations harbored recurrent oncogenic mutations in AKT1 (p.Glu17Lys) and SMO (p.Trp535Leu) and exhibited immunohistochemical evidence of activation of these pathways.
    explanation: Independent genomic sequencing supports AKT1 and SMO mutations in the non-NF2 meningioma subset.
  - reference: DOI:10.1038/ng.3651
    reference_title: Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
    explanation: This title-level cached reference supports POLR2A as a recurrent driver defining a distinct meningioma subset.
- name: CDKN2A/B Deletion-Associated Progression
  description: >-
    CDKN2A and CDKN2B deletion marks aggressive meningioma biology and is linked
    to shorter progression-free survival, with homozygous deletion incorporated
    into modern high-grade risk assessment.
  genes:
  - preferred_term: CDKN2A
    term:
      id: hgnc:1787
      label: CDKN2A
  - preferred_term: CDKN2B
    term:
      id: hgnc:1788
      label: CDKN2B
  evidence:
  - reference: DOI:10.1186/s40478-023-01690-y
    reference_title: 'CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data'
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression.
    explanation: Patient-level meta-analysis supports CDKN2A/B deletion as progression-associated meningioma biology.
  - reference: DOI:10.3390/cancers15112945
    reference_title: "Epigenetic, Genetic, and Transcriptomic Profiling of Meningiomas: Molecular Patterns Correlate with Clinical Prognosis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk.
    explanation: WHO-linked molecular criteria support CDKN2A/B homozygous deletion as a high-risk meningioma feature.
- name: TERT Promoter Mutation-Associated Grade 3 Biology
  description: >-
    TERT promoter mutations identify a molecularly high-risk meningioma subset
    associated with recurrence risk and WHO grade 3 assignment in modern
    classification.
  genes:
  - preferred_term: TERT
    term:
      id: hgnc:11730
      label: TERT
  evidence:
  - reference: DOI:10.3390/cancers15112945
    reference_title: "Epigenetic, Genetic, and Transcriptomic Profiling of Meningiomas: Molecular Patterns Correlate with Clinical Prognosis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk.
    explanation: This directly supports TERT promoter mutation status as a grade 3, recurrence-associated molecular feature.
- name: NOTCH3 Activation in Aggressive Meningioma
  description: >-
    NOTCH3 signaling is associated with aggressive meningioma behavior and links
    to downstream metabolic reprogramming.
  genes:
  - preferred_term: NOTCH3
    term:
      id: hgnc:7883
      label: NOTCH3
  downstream:
  - target: CD36-Associated Fatty Acid Oxidation Reprogramming
    description: NOTCH3-high states correlate with FAO-related metabolic features.
    evidence:
    - reference: PMID:40924320
      reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: "Single-cell RNA sequencing revealed a correlation with CD36, a key fatty acid transporter."
      explanation: Supports a direct link between NOTCH3-associated states and CD36-linked lipid metabolism.
  evidence:
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "While prior work has linked NOTCH3 expression to higher-grade meningiomas and treatment resistance, the metabolic phenotype of NOTCH3 activation remains unexplored in meningioma."
    explanation: Supports NOTCH3 as a marker of aggressive, treatment-resistant meningioma states.
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "We show that NOTCH3 mediates a metabolic shift towards fatty acid oxidation (FAO), depleting lipid availability and conferring resistance to ferroptosis."
    explanation: Directly links NOTCH3 activation to metabolic rewiring in aggressive meningioma models.
- name: CD36-Associated Fatty Acid Oxidation Reprogramming
  description: >-
    NOTCH3-associated meningioma states show increased CD36-linked fatty acid
    utilization and FAO-like mitochondrial metabolism.
  genes:
  - preferred_term: CD36
    term:
      id: hgnc:1663
      label: CD36
  biological_processes:
  - preferred_term: fatty acid transport
    modifier: INCREASED
    term:
      id: GO:0015908
      label: fatty acid transport
  - preferred_term: fatty acid beta-oxidation
    modifier: INCREASED
    term:
      id: GO:0006635
      label: fatty acid beta-oxidation
  downstream:
  - target: Ferroptosis Resistance
    description: Enhanced FAO-linked metabolism is associated with ferroptosis evasion.
    evidence:
    - reference: PMID:40924320
      reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: "We show that NOTCH3 mediates a metabolic shift towards fatty acid oxidation (FAO), depleting lipid availability and conferring resistance to ferroptosis."
      explanation: Supports the FAO to ferroptosis-resistance edge in aggressive meningioma models.
  evidence:
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "Single-cell RNA sequencing revealed a correlation with CD36, a key fatty acid transporter."
    explanation: Supports CD36-linked fatty acid substrate routing in NOTCH3-associated states.
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "NOTCH3 ICD overexpression (OE) exhibited depletion of fatty acid pools, alongside transcriptional upregulation of canonical FAO genes."
    explanation: Supports enhanced FAO transcriptional programming under NOTCH3 activation.
- name: Ferroptosis Resistance
  description: >-
    Aggressive meningioma cells with activated NOTCH3 signaling display reduced
    sensitivity to ferroptosis, indicating a potential mechanism of treatment
    resistance.
  biological_processes:
  - preferred_term: ferroptosis
    modifier: DECREASED
    term:
      id: GO:0097707
      label: ferroptosis
  evidence:
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "Additionally, NOTCH3 OE cells exhibit increased resistance to RSL3-induced ferroptosis, a phenotype that was reversed with CPT1 inhibition."
    explanation: The abstract reports ferroptosis resistance and partial reversibility with metabolic inhibition.
- name: NY-ESO-1-Associated Immune Vulnerability in Malignant Meningioma
  description: >-
    Higher NY-ESO-1 expression in malignant meningioma is linked to higher tumor
    grade and may expose a targetable immune vulnerability.
  genes:
  - preferred_term: CTAG1B
    term:
      id: hgnc:2491
      label: CTAG1B
  downstream:
  - target: NY-ESO-1-Dependent TCR-T Cytolysis Susceptibility
    description: Higher NY-ESO-1 expression increases susceptibility to NY-ESO-1-directed TCR-T killing.
    evidence:
    - reference: PMID:40802116
      reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: "CH157-HLA-A2.1 cells, with native high NY-ESO-1 expression, experienced > 60% and then nearly 100% cytolysis after co-culture with TCR-T for 10 and 24 h, respectively, compared with control T-cells (p < 0.0001)."
      explanation: Shows expression-dependent susceptibility to NY-ESO-1 TCR-T cytolysis.
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "NY-ESO-1 expression correlated with tumor grade (n = 35; p < 0.01)."
    explanation: Human tumor specimens show NY-ESO-1 tracks with higher-grade disease.
- name: NY-ESO-1-Dependent TCR-T Cytolysis Susceptibility
  description: >-
    Meningioma cells with higher NY-ESO-1 expression show stronger cytolytic
    response to NY-ESO-1-targeted TCR-transduced T cells.
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "CH157-HLA-A2.1 cells, with native high NY-ESO-1 expression, experienced > 60% and then nearly 100% cytolysis after co-culture with TCR-T for 10 and 24 h, respectively, compared with control T-cells (p < 0.0001)."
    explanation: Demonstrates expression-linked cytolytic sensitivity to NY-ESO-1-targeted TCR-T.
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: MODEL_ORGANISM
    snippet: "Systemic ACT results in significantly increased survival in vivo in high-grade meningioma."
    explanation: In vivo model data support therapeutic translation of NY-ESO-1-directed ACT.
genetic:
- name: NF2
  association: Recurrent tumor suppressor loss
  gene_term:
    preferred_term: NF2
    term:
      id: hgnc:7773
      label: NF2
  evidence:
  - reference: DOI:10.1158/1541-7786.MCR-12-0116
    reference_title: "Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Loss of the NF2 tumor suppressor gene is the most common genetic alteration in meningiomas, and the NF2 gene product, Merlin, acts upstream of the Hippo pathway.
    explanation: Establishes NF2 loss as a common meningioma genetic alteration.
- name: CDKN2A
  association: Homozygous or heterozygous deletion associated with progression
  gene_term:
    preferred_term: CDKN2A
    term:
      id: hgnc:1787
      label: CDKN2A
  evidence:
  - reference: DOI:10.1186/s40478-023-01690-y
    reference_title: 'CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data'
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression.
    explanation: Supports CDKN2A deletion as part of the progression-associated CDKN2A/B locus.
- name: CDKN2B
  association: Homozygous or heterozygous deletion associated with progression
  gene_term:
    preferred_term: CDKN2B
    term:
      id: hgnc:1788
      label: CDKN2B
  evidence:
  - reference: DOI:10.1186/s40478-023-01690-y
    reference_title: 'CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data'
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Both hetero- or homozygous CDKN2A/B deletions were significantly associated with shortened time to meningioma progression.
    explanation: Supports CDKN2B deletion as part of the progression-associated CDKN2A/B locus.
- name: TERT
  association: Promoter mutation associated with WHO grade 3 biology
  gene_term:
    preferred_term: TERT
    term:
      id: hgnc:11730
      label: TERT
  evidence:
  - reference: DOI:10.3390/cancers15112945
    reference_title: "Epigenetic, Genetic, and Transcriptomic Profiling of Meningiomas: Molecular Patterns Correlate with Clinical Prognosis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: The grading system established by the World Health Organization (WHO) has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3, owing to their association with increased recurrence risk.
    explanation: Supports TERT promoter mutation as a high-grade molecular criterion.
- name: TRAF7
  association: Recurrent non-NF2 driver mutation
  gene_term:
    preferred_term: TRAF7
    term:
      id: hgnc:20456
      label: TRAF7
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO.
    explanation: Supports TRAF7 as part of the recurrent non-NF2 driver mutation landscape.
- name: KLF4
  association: Recurrent non-NF2 driver mutation
  gene_term:
    preferred_term: KLF4
    term:
      id: hgnc:6348
      label: KLF4
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Mutations in TRAF7 commonly occurred with a recurrent mutation (K409Q) in KLF4, a transcription factor known for its role in inducing pluripotency, or with AKT1(E17K), a mutation known to activate the PI3K pathway.
    explanation: Supports recurrent KLF4 mutation as part of the non-NF2 meningioma driver landscape.
- name: AKT1
  association: Recurrent activating mutation in non-NF2 meningioma
  gene_term:
    preferred_term: AKT1
    term:
      id: hgnc:391
      label: AKT1
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: The known neoplasia-related recurrent mutation, AKT1E17K, was identified in 38 meningiomas.
    explanation: Supports AKT1 E17K as a recurrent meningioma driver mutation.
- name: SMO
  association: Recurrent Hedgehog-pathway driver mutation
  gene_term:
    preferred_term: SMO
    term:
      id: hgnc:11119
      label: SMO
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Finally, in 11 tumors, we identified mutations in SMO, which is expressed in meningiomas (fig. S5).
    explanation: Supports SMO as a recurrent non-NF2 meningioma driver.
- name: PIK3CA
  association: Less common PI3K-pathway alteration
  gene_term:
    preferred_term: PIK3CA
    term:
      id: hgnc:8975
      label: PIK3CA
  evidence:
  - reference: PMID:23348505
    reference_title: "Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: In addition, we identified single mutations in genes previously reported to play a role in other neoplasias, including CREBBP, PIK3CA (R108H variant), PIK3R1 (deletion p.306-307), and BRCA1 as well as two SMARCB1 mutations, which coexisted with NF2 loss and have previously been reported in meningiomas (4) (table S3).
    explanation: Supports PIK3CA as a less common but reported PI3K-pathway alteration in meningioma sequencing.
- name: POLR2A
  association: Recurrent somatic mutation defining a distinct non-NF2 subset
  gene_term:
    preferred_term: POLR2A
    term:
      id: hgnc:9187
      label: POLR2A
  evidence:
  - reference: DOI:10.1038/ng.3651
    reference_title: Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
    explanation: Title-level cached reference supports POLR2A as a recurrent meningioma mutation subgroup.
- name: NOTCH3
  association: Upregulated signaling in aggressive meningioma models
  gene_term:
    preferred_term: NOTCH3
    term:
      id: hgnc:7883
      label: NOTCH3
  evidence:
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "While prior work has linked NOTCH3 expression to higher-grade meningiomas and treatment resistance, the metabolic phenotype of NOTCH3 activation remains unexplored in meningioma."
    explanation: Supports NOTCH3 as an aggressive-disease-associated molecular feature.
- name: CD36
  association: Correlated with NOTCH3-high metabolic state
  gene_term:
    preferred_term: CD36
    term:
      id: hgnc:1663
      label: CD36
  evidence:
  - reference: PMID:40924320
    reference_title: "NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "Single-cell RNA sequencing revealed a correlation with CD36, a key fatty acid transporter."
    explanation: Supports CD36 as a linked metabolic mediator in aggressive meningioma cells.
- name: CTAG1B (NY-ESO-1)
  association: Higher expression in higher-grade malignant meningioma
  gene_term:
    preferred_term: CTAG1B
    term:
      id: hgnc:2491
      label: CTAG1B
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "NY-ESO-1 expression correlated with tumor grade (n = 35; p < 0.01)."
    explanation: Supports grade-associated CTAG1B/NY-ESO-1 biology in malignant meningioma.
biochemical:
- name: High NY-ESO-1 Nuclear Expression
  biomarker_term:
    preferred_term: NY-ESO-1
    term:
      id: NCIT:C39286
      label: Cancer/Testis Antigen 1
  presence: Increased in higher-grade malignant meningioma
  notes: Higher NY-ESO-1 expression is associated with poorer progression-free survival.
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "High NY-ESO-1 nuclear expression predicted a worse progression-free-survival (p = 0.0167)."
    explanation: Supports NY-ESO-1 expression as a prognostic molecular feature.
histopathology:
- name: Histopathologically Confirmed High-Grade Meningioma
  finding_term:
    preferred_term: meningioma
    term:
      id: NCIT:C3230
      label: Meningioma
  description: >-
    Recurrent/residual high-grade meningioma cohorts treated with SRS include
    histopathologically confirmed atypical or anaplastic tumors.
  context: recurrent or residual high-grade meningioma
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "All patients had undergone craniotomy and histopathologic confirmation of atypical or anaplastic meningioma."
    explanation: Confirms grade II/III pathology as a defining tissue-level feature in the studied cohort.
- name: Predominance of Atypical Histology in High-Grade Cohort
  finding_term:
    preferred_term: atypical meningioma
    term:
      id: NCIT:C4723
      label: Atypical Meningioma
  description: >-
    In a large recurrent/residual high-grade series, atypical histology (grade
    II) was substantially more frequent than anaplastic histology (grade III).
  context: recurrent or residual high-grade meningioma
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Atypical meningioma accounted for the vast majority of cases (105; 93.8%) with only 7 cases of anaplastic meningioma (6.2%)."
    explanation: Quantifies the high-grade histology distribution in a treated clinical cohort.
phenotypes:
- category: Oncologic
  name: High recurrence risk in high-grade disease
  description: >-
    WHO grade II and III meningiomas demonstrate substantial recurrence risk and
    require close longitudinal follow-up.
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "present therapeutic challenges due to their aggressive behavior and high risk of recurrence."
    explanation: The large retrospective cohort highlights recurrence as a defining high-grade phenotype.
- category: Oncologic
  name: Shorter progression-free survival with high NY-ESO-1 expression
  description: >-
    Higher NY-ESO-1 expression is associated with worse progression-free outcomes
    in malignant meningioma.
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "High NY-ESO-1 nuclear expression predicted a worse progression-free-survival (p = 0.0167)."
    explanation: The abstract provides a direct prognostic association between NY-ESO-1 and worse PFS.
- category: Neurologic
  name: Seizure at diagnosis
  frequency: OCCASIONAL
  description: A substantial subset of newly diagnosed meningioma patients present with seizures.
  phenotype_term:
    preferred_term: Seizure
    term:
      id: HP:0001250
      label: Seizure
  evidence:
  - reference: PMID:40645150
    reference_title: "Predicting epilepsy in patients diagnosed with intracranial meningiomas: A systematic review and meta-analysis of clinical and anatomical risk factors."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "new-onset seizures were reported by 26% of newly diagnosed meningioma patients."
    explanation: Meta-analysis quantifies seizure presentation at diagnosis.
- category: Neurologic
  name: Headache
  description: Headache is a common but non-specific symptom in patients with incidental meningioma.
  phenotype_term:
    preferred_term: Headache
    term:
      id: HP:0002315
      label: Headache
  evidence:
  - reference: PMID:40178655
    reference_title: "Prevalence and symptoms of incidental meningiomas: a population-based study."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Common and non-specific symptoms such as headache and dizziness may be wrongfully attributed to meningiomas, which can lead to unnecessary surgery and anxiety for the patient."
    explanation: Supports headache as a frequent, non-specific symptom context in incidental meningioma evaluation.
diagnosis:
- name: Multiparameter MRI-based differentiation from intracranial SFT
  description: >-
    Preoperative imaging-based differentiation between meningioma and intracranial
    solitary fibrous tumor remains clinically important and can be improved with
    integrated multiparameter MRI models.
  diagnosis_term:
    preferred_term: magnetic resonance imaging procedure
    term:
      id: MAXO:0000424
      label: magnetic resonance imaging procedure
  evidence:
  - reference: PMID:40625299
    reference_title: "Multiparameter MRI-based automatic segmentation and diagnostic models for the differentiation of intracranial solitary fibrous tumors and meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Their similar imaging features make preoperative differentiation difficult, resulting in high misdiagnosis rates."
    explanation: This captures the core diagnostic challenge in routine imaging assessment.
  - reference: PMID:40625299
    reference_title: "Multiparameter MRI-based automatic segmentation and diagnostic models for the differentiation of intracranial solitary fibrous tumors and meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The comprehensive system integrating automatic segmentation with diagnostic models can differentiate SFTs from meningiomas precisely."
    explanation: The study reports improved differential classification using integrated imaging models.
- name: WHO 2021 grade-informed prognostic stratification
  description: >-
    WHO 2021 grading and selected proliferative/imaging features stratify
    progression and survival risk in long-term follow-up cohorts.
  evidence:
  - reference: PMID:41094320
    reference_title: "Meningioma grade and molecular markers of proliferation, hypoxia, and vascularity as predictors of outcome in a cohort with long-term patient follow-up."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "PFS was independently associated with WHO 2021 grade 2 (HR 3.72, 95% CI 1.49-9.32), MIB-1 > 5% (HR 2.56, 95% CI 1.17-5.60), and PTBE ratio (HR 1.22 per 0.1 increment, 95% CI 1.04-1.43)."
    explanation: Supports grade-informed prognostic stratification in a long-term clinical cohort.
- name: Somatostatin receptor PET for extent and recurrence assessment
  description: >-
    SSTR-ligand PET can improve meningioma tissue detection and aid differential
    diagnosis, extent delineation, and recurrence-vs-scar assessment beyond
    structural MRI/CT alone.
  diagnosis_term:
    preferred_term: positron emission tomography procedure
    term:
      id: MAXO:0000137
      label: positron emission tomography procedure
  evidence:
  - reference: PMID:38898354
    reference_title: "Joint EANM/EANO/RANO/SNMMI practice guideline/procedure standards for diagnostics and therapy (theranostics) of meningiomas using radiolabeled somatostatin receptor ligands: version 1.0."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Positron emission tomography (PET) using somatostatin receptor (SSTR) ligands can detect meningioma tissue with high sensitivity and specificity and may provide clinically relevant information beyond that obtained from structural magnetic resonance imaging (MRI) or computed tomography (CT) imaging alone."
    explanation: Multi-society guideline supports added diagnostic value of SSTR PET.
epidemiology:
- name: Primary intracranial tumor frequency
  description: Meningiomas are the most frequent primary intracranial tumors.
  evidence:
  - reference: PMID:34618539
    reference_title: "Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Meningiomas are the most frequent primary intracranial tumors."
    explanation: Supports meningioma as the leading primary intracranial tumor category.
progression:
- phase: Clinical behavior spectrum
  notes: Clinical course ranges from indolent to aggressive and potentially fatal disease.
  evidence:
  - reference: PMID:34618539
    reference_title: "Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Patient outcome varies widely from benign to highly aggressive, ultimately fatal courses."
    explanation: Captures broad meningioma progression heterogeneity.
- phase: Post-SRS progression-free survival by grade
  notes: >-
    PFS declines with increasing WHO grade, with poorer outcomes in grade III
    recurrence.
  evidence:
  - reference: PMID:40940847
    reference_title: "Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "For WHO Grade I tumors, 3- to 5-year progression-free survival (PFS) ranged from 85% to 100%."
    explanation: Establishes favorable recurrent-disease PFS for grade I tumors after SRS.
  - reference: PMID:40940847
    reference_title: "Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Grade II meningiomas demonstrated more variable outcomes, with 3-year PFS ranging from 23% to 100%."
    explanation: Shows intermediate and heterogeneous grade II recurrence trajectory.
  - reference: PMID:40940847
    reference_title: "Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Grade III tumors had consistently poorer outcomes, with reported 1-year and 2-year PFS rates as low as 0% and 46%, respectively."
    explanation: Supports markedly worse progression trajectory in recurrent grade III disease.
- phase: Long-term prognostic covariates
  notes: Grade and extent of resection significantly influence long-term outcomes.
  evidence:
  - reference: PMID:41094320
    reference_title: "Meningioma grade and molecular markers of proliferation, hypoxia, and vascularity as predictors of outcome in a cohort with long-term patient follow-up."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "OS was associated with subtotal resection (HR 2.69, 95% CI 1.28-5.65) and WHO 2021 grade 2 (HR 4.27, 95% CI 1.61-11.33)."
    explanation: Supports resection extent and grade as major long-term survival determinants.
differential_diagnoses:
- name: Intracranial solitary fibrous tumor
  description: >-
    Intracranial SFT can mimic meningioma on imaging, making preoperative
    differentiation difficult.
  disease_term:
    preferred_term: solitary fibrous tumor
    term:
      id: MONDO:0016238
      label: solitary fibrous tumor
  distinguishing_features:
  - Multiparametric MRI-based segmentation and fusion models can improve preoperative discrimination between SFT and meningioma.
  evidence:
  - reference: PMID:40625299
    reference_title: "Multiparameter MRI-based automatic segmentation and diagnostic models for the differentiation of intracranial solitary fibrous tumors and meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Their similar imaging features make preoperative differentiation difficult, resulting in high misdiagnosis rates."
    explanation: Directly supports SFT as a clinically relevant differential diagnosis.
treatments:
- name: Gross total surgical resection
  description: >-
    Surgical intervention with tissue diagnosis remains central to definitive
    classification; gross total resection is frequently curative when feasible.
  treatment_term:
    preferred_term: surgical procedure
    term:
      id: MAXO:0000004
      label: surgical procedure
  evidence:
  - reference: PMID:34181733
    reference_title: "EANO guideline on the diagnosis and management of meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "A gross total surgical resection including the involved dura is often curative."
    explanation: EANO guideline supports gross total resection as a key curative-intent approach.
- name: Watch-and-scan surveillance for selected incidental tumors
  description: >-
    Asymptomatic or elderly patients with incidentally diagnosed meningioma may
    be managed conservatively with serial imaging surveillance.
  treatment_term:
    preferred_term: supportive care
    term:
      id: MAXO:0000950
      label: supportive care
  evidence:
  - reference: PMID:34181733
    reference_title: "EANO guideline on the diagnosis and management of meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "a significant proportion of meningiomas, notably in patients that are asymptomatic or elderly or both, may be managed by a watch-and-scan strategy."
    explanation: Guideline-endorsed conservative management option for selected patients.
- name: Repeated stereotactic radiosurgery (SRS)
  description: >-
    Repeated SRS is used for recurrent or residual high-grade meningioma and has
    a manageable toxicity profile in retrospective clinical experience.
  treatment_term:
    preferred_term: stereotactic radiosurgery
    term:
      id: MAXO:0009088
      label: stereotactic radiosurgery
  evidence:
  - reference: PMID:40802120
    reference_title: "Repeated stereotactic radiosurgery for high grade meningioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Our data suggest that SRS is a relatively safe and effective treatment option for recurrent or residual high-grade meningioma, with an acceptable complication profile, even when performed repeatedly."
    explanation: Human cohort data support repeated radiosurgery as a practical salvage option.
- name: Fractionated radiotherapy for inoperable or recurrent disease
  description: >-
    Fractionated RT is an accepted treatment option when tumors are inoperable
    or recur and require additional local control.
  treatment_term:
    preferred_term: radiation therapy
    term:
      id: MAXO:0000014
      label: radiation therapy
  evidence:
  - reference: PMID:34181733
    reference_title: "EANO guideline on the diagnosis and management of meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Inoperable or recurrent tumors requiring treatment can be treated with radiosurgery, if the size or the vicinity of critical structures allows that, or with fractionated radiotherapy (RT)."
    explanation: EANO guidance supports fractionated RT as a standard local modality in selected cases.
- name: NY-ESO-1 TCR-transduced T-cell therapy (investigational)
  description: >-
    Preclinical adoptive cell transfer using NY-ESO-1-directed TCR-T cells shows
    anti-tumor activity and survival benefit in high-grade meningioma models.
  treatment_term:
    preferred_term: cellular therapy
    term:
      id: MAXO:0000016
      label: cellular therapy
  target_mechanisms:
  - target: NY-ESO-1-Associated Immune Vulnerability in Malignant Meningioma
    treatment_effect: INHIBITS
    description: NY-ESO-1-targeted TCR-T cells reduce viability of NY-ESO-1-high malignant meningioma cells.
    evidence:
    - reference: PMID:40802116
      reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: "CH157-HLA-A2.1 cells, with native high NY-ESO-1 expression, experienced > 60% and then nearly 100% cytolysis after co-culture with TCR-T for 10 and 24 h, respectively, compared with control T-cells (p < 0.0001)."
      explanation: Supports mechanism-linked killing of NY-ESO-1-high malignant meningioma cells by TCR-T.
  evidence:
  - reference: PMID:40802116
    reference_title: "Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells."
    supports: SUPPORT
    evidence_source: MODEL_ORGANISM
    snippet: "Systemic ACT results in significantly increased survival in vivo in high-grade meningioma."
    explanation: In vivo preclinical data support NY-ESO-1-directed ACT as a candidate strategy.
datasets:
- accession: geo:GSE292327
  title: Development, validation, and clinical utility of a novel methylation classifier for recurrence risk prediction in meningiomas
  description: >-
    DNA methylation profiling cohort used to develop and validate recurrence
    risk prediction models for meningioma.
  organism:
    preferred_term: human
    term:
      id: NCBITaxon:9606
      label: Homo sapiens
  data_type: METHYLATION
  sample_types:
  - preferred_term: meningioma tissue
    term:
      id: UBERON:0010506
      label: meningeal dura mater
    tissue_term:
      preferred_term: meningeal dura
      term:
        id: UBERON:0010506
        label: meningeal dura mater
  sample_count: 223
  conditions:
  - meningioma
  - recurrence risk stratification
  publication: PMID:41466325
  notes: GEO metadata indicates a clinically validated recurrence-risk methylation classifier cohort.
  evidence:
  - reference: PMID:41466325
    reference_title: "Development, validation, and utility of a clinically applicable methylation classifier for recurrence risk prediction in meningiomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Using samples from 217 patients, we developed, validated, and implemented a clinically applicable methylation classifier for prognostic stratification of meningiomas based on k-means clustering of methylation data."
    explanation: Supports the recurrence-risk methylation stratification intent of this dataset entry.
- accession: geo:GSE313693
  title: Single-nuclei RNA-seq of meningioma
  description: >-
    Single-nuclei transcriptomic dataset used to characterize meningioma
    microenvironmental states and refine subtype/risk continuum analyses.
  organism:
    preferred_term: human
    term:
      id: NCBITaxon:9606
      label: Homo sapiens
  data_type: SINGLE_CELL_RNA_SEQ
  sample_types:
  - preferred_term: meningioma tissue nuclei
    term:
      id: UBERON:0010506
      label: meningeal dura mater
    tissue_term:
      preferred_term: meningeal dura
      term:
        id: UBERON:0010506
        label: meningeal dura mater
  sample_count: 26
  conditions:
  - meningioma
  - microenvironment profiling
  publication: PMID:41663806
  notes: GEO metadata reports single-nuclei RNA sequencing for meningioma subtype refinement.
  evidence:
  - reference: PMID:41663806
    reference_title: "A microenvironment-determined risk continuum refines subtyping in meningioma and reveals determinants of machine learning-based tumor classification."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Here, by applying multiomic profiling and multiple lines of orthogonal computational evaluation in multiple independent datasets, we found that not only tumor cell characteristics but also incremental changes in the tumor microenvironment (TME) have impact on epigenetic meningioma classification and clinical outcome."
    explanation: Supports multiomic dataset use for microenvironment-linked meningioma classification refinement.
references:
- reference: DOI:10.1158/1541-7786.MCR-12-0116
  title: Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas
  findings: []
- reference: PMID:23348505
  title: Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO.
  findings: []
- reference: PMID:23334667
  title: Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations.
  findings: []
- reference: DOI:10.1038/ng.3651
  title: Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
  findings: []
- reference: PMID:40625299
  title: Multiparameter MRI-based automatic segmentation and diagnostic models for the differentiation of intracranial solitary fibrous tumors and meningiomas.
  findings: []
- reference: PMID:40802116
  title: Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells.
  findings: []
- reference: PMID:40924320
  title: NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas.
  findings: []
- reference: PMID:40802120
  title: Repeated stereotactic radiosurgery for high grade meningioma.
  findings: []
- reference: PMID:34618539
  title: "Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated."
  findings: []
- reference: PMID:40940847
  title: "Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches."
  findings: []
- reference: PMID:41094320
  title: "Meningioma grade and molecular markers of proliferation, hypoxia, and vascularity as predictors of outcome in a cohort with long-term patient follow-up."
  findings: []
- reference: PMID:40178655
  title: "Prevalence and symptoms of incidental meningiomas: a population-based study."
  findings: []
- reference: PMID:40645150
  title: "Predicting epilepsy in patients diagnosed with intracranial meningiomas: A systematic review and meta-analysis of clinical and anatomical risk factors."
  findings: []
- reference: PMID:34181733
  title: EANO guideline on the diagnosis and management of meningiomas.
  findings: []
- reference: PMID:38898354
  title: "Joint EANM/EANO/RANO/SNMMI practice guideline/procedure standards for diagnostics and therapy (theranostics) of meningiomas using radiolabeled somatostatin receptor ligands: version 1.0."
  findings: []
- reference: PMID:41466325
  title: Development, validation, and utility of a clinically applicable methylation classifier for recurrence risk prediction in meningiomas.
  findings: []
- reference: PMID:41663806
  title: A microenvironment-determined risk continuum refines subtyping in meningioma and reveals determinants of machine learning-based tumor classification.
  findings: []
- reference: DOI:10.1002/cncr.35279
  title: Evolving concepts in meningioma management in the era of genomics
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningioma is the most common type of primary brain tumor.
    supporting_text: Meningioma is the most common type of primary brain tumor.
    evidence:
    - reference: DOI:10.1002/cncr.35279
      reference_title: Evolving concepts in meningioma management in the era of genomics
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Meningioma is the most common type of primary brain tumor.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1007/978-3-031-29750-2_11
  title: Genomic Landscape of Meningiomas
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Genomic Landscape of Meningiomas
    supporting_text: Genomic Landscape of Meningiomas
- reference: DOI:10.1007/s00701-022-05301-y
  title: 'The WHO 2021 Classification of Central Nervous System tumours: a practical update on what neurosurgeons need to know—a minireview'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours.
    supporting_text: The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours.
    evidence:
    - reference: DOI:10.1007/s00701-022-05301-y
      reference_title: 'The WHO 2021 Classification of Central Nervous System tumours: a practical update on what neurosurgeons need to know—a minireview'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1007/s11060-023-04253-2
  title: 'The multiomic landscape of meningiomas: a review and update'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningiomas are the most common primary brain tumor in adults.
    supporting_text: Meningiomas are the most common primary brain tumor in adults.
    evidence:
    - reference: DOI:10.1007/s11060-023-04253-2
      reference_title: 'The multiomic landscape of meningiomas: a review and update'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Meningiomas are the most common primary brain tumor in adults.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1038/s41591-024-03167-4
  title: Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma
    supporting_text: Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma
- reference: DOI:10.1093/jncics/pkab035
  title: 'Incidence of Benign Meningiomas in the United States: Current and Future Trends'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades.
    supporting_text: Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades.
    evidence:
    - reference: DOI:10.1093/jncics/pkab035
      reference_title: 'Incidence of Benign Meningiomas in the United States: Current and Future Trends'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1093/neuonc/noab106
  title: 'The 2021 WHO Classification of Tumors of the Central Nervous System: a summary'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
    supporting_text: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
    evidence:
    - reference: DOI:10.1093/neuonc/noab106
      reference_title: 'The 2021 WHO Classification of Tumors of the Central Nervous System: a summary'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1093/neuonc/noae082
  title: 'Meningioma: International Consortium on Meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging.
    supporting_text: Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging.
    evidence:
    - reference: DOI:10.1093/neuonc/noae082
      reference_title: 'Meningioma: International Consortium on Meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1093/neuonc/noae145
  title: 'CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017–2021'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: 'CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017–2021'
    supporting_text: The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population.
    evidence:
    - reference: DOI:10.1093/neuonc/noae145
      reference_title: 'CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017–2021'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1097/cm9.0000000000002391
  title: 'Molecular diagnosis and treatment of meningiomas: an expert consensus (2022)'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations.
    supporting_text: Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations.
    evidence:
    - reference: DOI:10.1097/cm9.0000000000002391
      reference_title: 'Molecular diagnosis and treatment of meningiomas: an expert consensus (2022)'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1186/s40478-023-01690-y
  title: 'CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas.
    supporting_text: Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas.
    evidence:
    - reference: DOI:10.1186/s40478-023-01690-y
      reference_title: 'CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.1186/s40478-024-01739-6
  title: Clinical implications of DNA methylation-based integrated classification of histologically defined grade 2 meningiomas
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas.
    supporting_text: The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas.
    evidence:
    - reference: DOI:10.1186/s40478-024-01739-6
      reference_title: Clinical implications of DNA methylation-based integrated classification of histologically defined grade 2 meningiomas
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.3390/cancers15112945
  title: 'Meningioma Grading beyond Histopathology: Relevance of Epigenetic and Genetic Features to Predict Clinical Outcome'
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningiomas are common tumors of the central nervous system.
    supporting_text: Meningiomas are common tumors of the central nervous system.
    evidence:
    - reference: DOI:10.3390/cancers15112945
      reference_title: 'Meningioma Grading beyond Histopathology: Relevance of Epigenetic and Genetic Features to Predict Clinical Outcome'
      supports: SUPPORT
      evidence_source: COMPUTATIONAL
      snippet: Meningiomas are common tumors of the central nervous system.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.3390/cancers16111978
  title: Surgical Management of High-Grade Meningiomas
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas.
    supporting_text: Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas.
    evidence:
    - reference: DOI:10.3390/cancers16111978
      reference_title: Surgical Management of High-Grade Meningiomas
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
- reference: DOI:10.3390/ijms25179631
  title: The Molecular and Immunological Landscape of Meningiomas
  found_in:
  - Meningioma-deep-research-falcon.md
  findings:
  - statement: Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature.
    supporting_text: Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature.
    evidence:
    - reference: DOI:10.3390/ijms25179631
      reference_title: The Molecular and Immunological Landscape of Meningiomas
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature.
      explanation: Deep research cited this publication as relevant literature for Meningioma.
📚

References & Deep Research

References

32
DOI:10.1158/1541-7786.MCR-12-0116
Yes-Associated Protein 1 Is Activated and Functions as an Oncogene in Meningiomas
No top-level findings curated for this source.
PMID:23348505
Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO.
No top-level findings curated for this source.
PMID:23334667
Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations.
No top-level findings curated for this source.
DOI:10.1038/ng.3651
Recurrent somatic mutations in POLR2A define a distinct subset of meningiomas
No top-level findings curated for this source.
PMID:40625299
Multiparameter MRI-based automatic segmentation and diagnostic models for the differentiation of intracranial solitary fibrous tumors and meningiomas.
No top-level findings curated for this source.
PMID:40802116
Immunotherapeutic targeting of NY-ESO-1 in malignant meningiomas with TCR-transduced T-cells.
No top-level findings curated for this source.
PMID:40924320
NOTCH3 drives fatty acid oxidation and ferroptosis resistance in aggressive meningiomas.
No top-level findings curated for this source.
PMID:40802120
Repeated stereotactic radiosurgery for high grade meningioma.
No top-level findings curated for this source.
PMID:34618539
Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated.
No top-level findings curated for this source.
PMID:40940847
Stereotactic Radiosurgery for Recurrent Meningioma: A Systematic Review of Risk Factors and Management Approaches.
No top-level findings curated for this source.
PMID:41094320
Meningioma grade and molecular markers of proliferation, hypoxia, and vascularity as predictors of outcome in a cohort with long-term patient follow-up.
No top-level findings curated for this source.
PMID:40178655
Prevalence and symptoms of incidental meningiomas: a population-based study.
No top-level findings curated for this source.
PMID:40645150
Predicting epilepsy in patients diagnosed with intracranial meningiomas: A systematic review and meta-analysis of clinical and anatomical risk factors.
No top-level findings curated for this source.
PMID:34181733
EANO guideline on the diagnosis and management of meningiomas.
No top-level findings curated for this source.
PMID:38898354
Joint EANM/EANO/RANO/SNMMI practice guideline/procedure standards for diagnostics and therapy (theranostics) of meningiomas using radiolabeled somatostatin receptor ligands: version 1.0.
No top-level findings curated for this source.
PMID:41466325
Development, validation, and utility of a clinically applicable methylation classifier for recurrence risk prediction in meningiomas.
No top-level findings curated for this source.
PMID:41663806
A microenvironment-determined risk continuum refines subtyping in meningioma and reveals determinants of machine learning-based tumor classification.
No top-level findings curated for this source.
DOI:10.1002/cncr.35279
Evolving concepts in meningioma management in the era of genomics
1 finding
Meningioma is the most common type of primary brain tumor.
"Meningioma is the most common type of primary brain tumor."
Show evidence (1 reference)
DOI:10.1002/cncr.35279 SUPPORT Human Clinical
"Meningioma is the most common type of primary brain tumor."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1007/978-3-031-29750-2_11
Genomic Landscape of Meningiomas
1 finding
Genomic Landscape of Meningiomas
"Genomic Landscape of Meningiomas"
DOI:10.1007/s00701-022-05301-y
The WHO 2021 Classification of Central Nervous System tumours: a practical update on what neurosurgeons need to know—a minireview
1 finding
The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours.
"The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours."
Show evidence (1 reference)
DOI:10.1007/s00701-022-05301-y SUPPORT Other
"The World Health Organization (WHO) Classification of Tumours, also known as WHO Blue Books, represents an international standardised tool in the diagnostic work-up of tumours."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1007/s11060-023-04253-2
The multiomic landscape of meningiomas: a review and update
1 finding
Meningiomas are the most common primary brain tumor in adults.
"Meningiomas are the most common primary brain tumor in adults."
Show evidence (1 reference)
DOI:10.1007/s11060-023-04253-2 SUPPORT Other
"Meningiomas are the most common primary brain tumor in adults."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1038/s41591-024-03167-4
Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma
1 finding
Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma
"Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma"
DOI:10.1093/jncics/pkab035
Incidence of Benign Meningiomas in the United States: Current and Future Trends
1 finding
Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades.
"Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades."
Show evidence (1 reference)
DOI:10.1093/jncics/pkab035 SUPPORT Other
"Benign meningiomas are the most frequently reported central nervous system tumors in the United States, with increasing incidence in past decades."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1093/neuonc/noab106
The 2021 WHO Classification of Tumors of the Central Nervous System: a summary
1 finding
The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
"The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors."
Show evidence (1 reference)
DOI:10.1093/neuonc/noab106 SUPPORT Other
"The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1093/neuonc/noae082
Meningioma: International Consortium on Meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients
1 finding
Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging.
"Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging."
Show evidence (1 reference)
DOI:10.1093/neuonc/noae082 SUPPORT Other
"Meningiomas are the most common primary intracranial tumors in adults and are increasing in incidence due to the aging population and increased access to neuroimaging."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1093/neuonc/noae145
CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017–2021
1 finding
CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017–2021
"The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in..."
Show evidence (1 reference)
DOI:10.1093/neuonc/noae145 SUPPORT Human Clinical
"The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in..."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1097/cm9.0000000000002391
Molecular diagnosis and treatment of meningiomas: an expert consensus (2022)
1 finding
Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations.
"Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations."
Show evidence (1 reference)
DOI:10.1097/cm9.0000000000002391 SUPPORT Other
"Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1186/s40478-023-01690-y
CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data
1 finding
Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas.
"Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas."
Show evidence (1 reference)
DOI:10.1186/s40478-023-01690-y SUPPORT Other
"Homozygous CDKN2A/B deletion has been associated with an increased risk of recurrence in meningiomas."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.1186/s40478-024-01739-6
Clinical implications of DNA methylation-based integrated classification of histologically defined grade 2 meningiomas
1 finding
The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas.
"The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas."
Show evidence (1 reference)
DOI:10.1186/s40478-024-01739-6 SUPPORT Human Clinical
"The combination of DNA methylation analysis with histopathological and genetic features allows for a more accurate risk stratification and classification of meningiomas."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.3390/cancers15112945
Meningioma Grading beyond Histopathology: Relevance of Epigenetic and Genetic Features to Predict Clinical Outcome
1 finding
Meningiomas are common tumors of the central nervous system.
"Meningiomas are common tumors of the central nervous system."
Show evidence (1 reference)
DOI:10.3390/cancers15112945 SUPPORT Computational
"Meningiomas are common tumors of the central nervous system."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.3390/cancers16111978
Surgical Management of High-Grade Meningiomas
1 finding
Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas.
"Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas."
Show evidence (1 reference)
DOI:10.3390/cancers16111978 SUPPORT Human Clinical
"Maximal resection with the preservation of neurological function are the mainstays of the surgical management of high-grade meningiomas."
Deep research cited this publication as relevant literature for Meningioma.
DOI:10.3390/ijms25179631
The Molecular and Immunological Landscape of Meningiomas
1 finding
Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature.
"Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature."
Show evidence (1 reference)
DOI:10.3390/ijms25179631 SUPPORT Human Clinical
"Meningiomas are the most common primary intracranial tumors in adults and typically have a slow-growing and benign nature."
Deep research cited this publication as relevant literature for Meningioma.

Deep Research

1
Falcon ▸
Disease Characteristics Research Template
Edison Scientific Literature 56 citations 2026-05-09T12:23:22.829197

Question: You are an expert researcher providing comprehensive, well-cited information.

Provide detailed information focusing on: 1. Key concepts and definitions with current understanding 2. Recent developments and latest research (prioritize 2023-2024 sources) 3. Current applications and real-world implementations 4. Expert opinions and analysis from authoritative sources 5. Relevant statistics and data from recent studies

Format as a comprehensive research report with proper citations. Include URLs and publication dates where available. Always prioritize recent, authoritative sources and provide specific citations for all major claims.

Disease Characteristics Research Template

Target Disease

  • Disease Name: Meningioma
  • MONDO ID: (if available)
  • Category: Neoplastic

Research Objectives

Please provide a comprehensive research report on Meningioma covering all of the disease characteristics listed below. This report will be used to populate a disease knowledge base entry. Be thorough and cite primary literature (PMID preferred) for all claims.

For each section, suggested databases/resources are listed. These are the first places you should search for information on each topic.

1. Disease Information

Search first: OMIM, Orphanet, ICD-10/ICD-11, MeSH, PubMed

  • What is the disease? Provide a concise overview.
  • What are the key identifiers? (OMIM, Orphanet, ICD-10/ICD-11, MeSH, Mondo)
  • What are the common synonyms and alternative names?
  • Is the information derived from individual patients (e.g., EHR) or aggregated disease-level resources?

2. Etiology

  • Disease Causal Factors: What are the primary causes? (genetic, environmental, infectious, mechanistic)
  • Risk Factors:

    Search first: PubMed, Cochrane Library, UpToDate, clinical guidelines, ClinVar, ClinGen, GWAS Catalog, PheGenI, CTD, CDC, WHO, epidemiological databases

  • Genetic risk factors (causal variants, susceptibility loci, modifier genes)
  • Environmental risk factors (toxins, lifestyle, occupational exposures, age, sex, family history)
  • Protective Factors:

    Search first: PubMed, Cochrane Library, clinical trial databases, GWAS Catalog, gnomAD, WHO, CDC, nutrition databases

  • Genetic protective factors (protective variants, modifier alleles)
  • Environmental protective factors (diet, lifestyle, exposures that reduce risk)
  • Gene-Environment Interactions: How do genetic and environmental factors interact to influence disease?

    Search first: CTD, PubMed, PheGenI, GxE databases

3. Phenotypes

Search first: HPO (Human Phenotype Ontology), OMIM, Orphanet, PubMed, clinicaltrials.gov, MedDRA, SNOMED CT, DECIPHER, LOINC

For each phenotype, provide: - Phenotype type: symptoms, clinical signs, physical manifestations, behavioral changes, or laboratory abnormalities

For symptoms/signs: HPO, OMIM, Orphanet, PubMed For behavioral changes: HPO, DSM, RDoC (Research Domain Criteria), PubMed For laboratory abnormalities: LOINC, SNOMED CT, LabTests Online, PubMed - Phenotype characteristics: Search first: OMIM, Orphanet, HPO, PubMed - Age of symptom onset (neonatal, childhood, adult-onset, late-onset) - Symptom severity (mild, moderate, severe, variable) - Symptom progression (stable, progressive, episodic, fluctuating) - Frequency among affected individuals (percentage or qualitative) - Quality of life impact: Effects on daily functioning and well-being (per-phenotype when possible) Search first: EQ-5D database, SF-36, WHO QOL databases, PubMed - Suggest HPO (Human Phenotype Ontology) terms for each phenotype

4. Genetic/Molecular Information

  • Causal Genes: Gene mutations or chromosomal abnormalities responsible for disease (gene symbols, OMIM IDs)

    Search first: OMIM, ClinVar, HGMD, Ensembl, NCBI Gene

  • Pathogenic Variants:
  • Affected genes (gene symbols, HGNC IDs) > Search first: OMIM, NCBI Gene, Ensembl, HGNC, UniProt, GeneCards
  • Variant classification (pathogenic, likely pathogenic, VUS per ACMG/AMP guidelines) > Search first: ClinVar, ClinGen, ACMG/AMP guidelines, VarSome
  • Variant type/class (missense, frameshift, nonsense, splice-site, structural)
  • Allele frequency in population databases > Search first: gnomAD, 1000 Genomes, ExAC, TOPMed, dbSNP
  • Somatic vs germline origin > Search first: COSMIC (somatic), ClinVar, ICGC, TCGA
  • Functional consequences (loss of function, gain of function, dominant negative)
  • Modifier Genes: Genes that modify disease severity or expression
  • Epigenetic Information: DNA methylation, histone modifications, chromatin changes affecting disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Chromosomal Abnormalities: Large-scale genetic changes (aneuploidy, translocations, inversions)

    Search first: DECIPHER, ClinVar, ECARUCA, UCSC Genome Browser

5. Environmental Information

  • Environmental Factors: Non-genetic contributing factors (toxins, radiation, pollution, occupational exposure)

    Search first: CTD (Comparative Toxicogenomics Database), TOXNET, PubMed, EPA databases

  • Lifestyle Factors: Behavioral factors (smoking, diet, exercise, alcohol consumption)

    Search first: CDC databases, WHO, PubMed, NHANES

  • Infectious Agents: If applicable, pathogens causing or triggering disease (bacteria, viruses, fungi, parasites)

    Search first: NCBI Taxonomy, ViPR, BV-BRC, MicrobeDB, GIDEON

6. Mechanism / Pathophysiology

  • Molecular Pathways: Specific signaling cascades or biochemical pathways involved (Wnt, MAPK, mTOR, PI3K-AKT, etc.)

    Search first: KEGG, Reactome, WikiPathways, PathBank, BioCyc

  • Cellular Processes: Cell-level mechanisms (apoptosis, autophagy, cell cycle dysregulation, inflammation, etc.)

    Search first: Gene Ontology (GO), Reactome, KEGG, PubMed

  • Protein Dysfunction: How protein structure or function is altered (misfolding, aggregation, loss of function, gain of function)

    Search first: UniProt, PDB (Protein Data Bank), InterPro, Pfam, AlphaFold

  • Metabolic Changes: Alterations in metabolic processes (energy metabolism, lipid metabolism, amino acid metabolism)

    Search first: KEGG, BioCyc, HMDB (Human Metabolome Database), BRENDA

  • Immune System Involvement: Role of immune response (autoimmunity, immunodeficiency, chronic inflammation)

    Search first: ImmPort, Immunome Database, IEDB, Gene Ontology

  • Tissue Damage Mechanisms: How tissues/ are injured (oxidative stress, ischemia, fibrosis, necrosis)

    Search first: PubMed, Gene Ontology, Reactome

  • Biochemical Abnormalities: Specific molecular defects (enzyme deficiencies, receptor dysfunction, ion channel defects)

    Search first: BRENDA, UniProt, KEGG, OMIM, PubMed

  • Epigenetic Changes: DNA methylation, histone modifications affecting gene expression in disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Molecular Profiling (if available):
  • Transcriptomics/gene expression changes > Search first: GEO (Gene Expression Omnibus), ArrayExpress, GTEx, Human Cell Atlas, SRA
  • Proteomics findings > Search first: PRIDE, ProteomeXchange, Human Protein Atlas, STRING, BioGRID
  • Metabolomics signatures > Search first: MetaboLights, Metabolomics Workbench, HMDB, METLIN
  • Lipidomics alterations > Search first: LIPID MAPS, SwissLipids, LipidHome, Metabolomics Workbench
  • Genomic structural features > Search first: UCSC Genome Browser, Ensembl, NCBI, dbVar, DGV
  • Advanced Technologies (if applicable):
  • Single-cell analysis findings (cell-type specific mechanisms, cellular heterogeneity) > Search first: Human Cell Atlas, Single Cell Portal, GEO, CELLxGENE
  • Spatial transcriptomics findings > Search first: GEO, Spatial Research, Vizgen, 10x Genomics data
  • Multi-omics integration results > Search first: TCGA, ICGC, cBioPortal, LinkedOmics, PubMed
  • Functional genomics screens (CRISPR, RNAi) > Search first: DepMap, GenomeRNAi, PubMed, BioGRID ORCS

For each mechanism, describe: - The causal chain from initial trigger to clinical manifestation - Which mechanisms are upstream vs downstream - What cell types and biological processes are involved - Suggest GO terms for biological processes and CL terms for cell types

7. Anatomical Structures Affected

  • Organ Level:
  • Primary organs directly affected
  • Secondary organ involvement (complications, secondary effects)
  • Body systems involved (cardiovascular, nervous, digestive, respiratory, endocrine, etc.)

    Search first: Uberon, FMA (Foundational Model of Anatomy), OMIM, HPO, ICD-11, MeSH, SNOMED CT

  • Tissue and Cell Level:
  • Specific tissue types affected (epithelial, connective, muscle, nervous)
  • Specific cell populations targeted (with Cell Ontology terms)

    Search first: Uberon, Human Protein Atlas, Cell Ontology, Human Cell Atlas, CellMarker, PanglaoDB

  • Subcellular Level:
  • Cellular compartments involved (mitochondria, nucleus, ER, lysosomes) (with GO Cellular Component terms)

    Search first: Gene Ontology (Cellular Component), UniProt, Human Protein Atlas

  • Localization:
  • Specific anatomical sites (with UBERON terms) > Search first: FMA, Uberon, NeuroNames (for brain), SNOMED CT
  • Lateralization (unilateral, bilateral, asymmetric) > Search first: HPO, clinical literature, imaging databases

8. Temporal Development

  • Onset:
  • Typical age of onset (congenital, pediatric, adult, geriatric)
  • Onset pattern (acute, subacute, chronic, insidious)

    Search first: OMIM, Orphanet, HPO, PubMed

  • Progression:
  • Disease stages (early, intermediate, advanced, end-stage) > Search first: Cancer Staging Manual (AJCC), WHO classifications, PubMed
  • Progression rate (rapid, slow, variable)
  • Disease course pattern (episodic, relapsing-remitting, progressive, stable)
  • Disease duration (self-limited, chronic lifelong)

    Search first: Disease registries, longitudinal cohort databases, natural history studies, PubMed, Orphanet, OMIM

  • Patterns:
  • Remission patterns (spontaneous, treatment-induced) > Search first: Clinical trial databases, disease registries, PubMed
  • Critical periods (time windows of vulnerability or opportunity for intervention) > Search first: PubMed, developmental biology databases, clinical guidelines

9. Inheritance and Population

  • Epidemiology:
  • Prevalence (cases per 100,000 at given time)
  • Incidence (new cases per 100,000 per year)

    Search first: Orphanet, CDC, WHO, GBD (Global Burden of Disease), national registries, SEER, disease registries

  • For Genetic Etiology:
  • Inheritance pattern (AD, AR, X-linked, mitochondrial, multifactorial, polygenic) > Search first: OMIM, Orphanet, ClinVar, GTR (Genetic Testing Registry)
  • Penetrance (complete, incomplete, age-dependent) > Search first: ClinVar, OMIM, PubMed, ClinGen
  • Expressivity (variable, consistent) > Search first: OMIM, ClinVar, PubMed
  • Genetic anticipation (increasing severity in successive generations) > Search first: OMIM, PubMed (especially for repeat expansion disorders)
  • Germline mosaicism > Search first: ClinVar, OMIM, genetic counseling literature, PubMed
  • Founder effects (population-specific mutations) > Search first: gnomAD, population genetics databases, PubMed
  • Consanguinity role > Search first: OMIM, population studies, genetic counseling resources
  • Carrier frequency > Search first: gnomAD, carrier screening databases, GeneReviews, GTR
  • Population Demographics:
  • Affected populations (ethnic or demographic groups with higher prevalence) > Search first: gnomAD, 1000 Genomes, PAGE Study, PubMed, population registries
  • Geographic distribution (endemic areas, regional variation) > Search first: WHO, CDC, GBD, Orphanet, geographic epidemiology databases
  • Geographic distribution of specific variants
  • Sex ratio (male:female) > Search first: Disease registries, OMIM, PubMed, epidemiological databases
  • Age distribution of affected individuals > Search first: CDC, disease registries, SEER, Orphanet

10. Diagnostics

  • Clinical Tests:
  • Laboratory tests (blood, urine, tissue chemistry, specific enzyme assays) > Search first: LOINC, LabTests Online, PubMed
  • Biomarkers (proteins, metabolites, genetic markers, circulating biomarkers) > Search first: FDA Biomarker List, BEST (Biomarkers, EndpointS, and other Tools), PubMed
  • Imaging studies (X-ray, CT, MRI, PET, ultrasound) > Search first: RadLex, DICOM, Radiopaedia, imaging databases
  • Functional tests (pulmonary function, cardiac stress tests) > Search first: LOINC, clinical guidelines, PubMed
  • Electrophysiology (EEG, EMG, ECG, nerve conduction studies) > Search first: LOINC, clinical neurophysiology databases, PubMed
  • Biopsy findings (histopathology, immunohistochemistry) > Search first: SNOMED CT, College of American Pathologists resources, PubMed
  • Pathology findings (microscopic examination) > Search first: SNOMED CT, Digital Pathology databases, PubMed
  • Genetic Testing:

    Search first: GTR (Genetic Testing Registry), GeneReviews, ClinGen

  • Overview of recommended genetic testing approach
  • Whole genome sequencing (WGS) utility > Search first: GTR, ClinVar, GEL (Genomics England), gnomAD
  • Whole exome sequencing (WES) utility > Search first: GTR, ClinVar, OMIM, GeneMatcher
  • Gene panels (which panels, which genes) > Search first: GTR, ClinVar, laboratory-specific databases
  • Single gene testing > Search first: GTR, ClinVar, OMIM, GeneReviews
  • Chromosomal microarray (CMA) > Search first: DECIPHER, ClinVar, dbVar, ECARUCA
  • Karyotyping > Search first: Chromosome Abnormality Database, ClinVar, cytogenetics resources
  • FISH > Search first: ClinVar, cytogenetics databases, PubMed
  • Mitochondrial DNA testing > Search first: MITOMAP, MSeqDR, ClinVar, GTR
  • Repeat expansion testing > Search first: GTR, ClinVar, repeat expansion databases, PubMed
  • Omics-Based Diagnostics (if applicable):
  • RNA sequencing / transcriptomics > Search first: GEO, ArrayExpress, GTEx, RNA-seq databases
  • Proteomics > Search first: PRIDE, ProteomeXchange, FDA Biomarker database
  • Metabolomics > Search first: MetaboLights, Metabolomics Workbench, HMDB
  • Epigenomics > Search first: GEO, ENCODE, Roadmap Epigenomics, MethBase
  • Liquid biopsy > Search first: COSMIC, ClinVar, liquid biopsy databases, PubMed
  • Clinical Criteria:
  • Standardized diagnostic criteria (DSM, ICD, society guidelines) > Search first: DSM-5, ICD-11, clinical society guidelines, UpToDate
  • Differential diagnosis (other conditions to rule out, with distinguishing features) > Search first: DynaMed, UpToDate, clinical decision support systems
  • Screening:
  • Screening methods for asymptomatic individuals (newborn screening, carrier screening, cascade screening) > Search first: ACMG recommendations, CDC newborn screening, GTR

11. Outcome/Prognosis

  • Survival and Mortality:
  • Survival rate (5-year, 10-year, overall) > Search first: SEER, cancer registries, disease-specific registries, PubMed
  • Life expectancy (with and without treatment if applicable) > Search first: Orphanet, disease registries, actuarial databases, PubMed
  • Mortality rate > Search first: CDC, WHO, GBD, national mortality databases
  • Disease-specific mortality (deaths directly attributable to disease) > Search first: Disease registries, CDC Wonder, GBD, PubMed
  • Morbidity and Function:
  • Morbidity (disease-related disability and health impacts) > Search first: GBD, WHO, disability databases, PubMed
  • Disability outcomes (long-term functional impairments) > Search first: ICF (International Classification of Functioning), disability registries
  • Quality of life measures (EQ-5D, SF-36, PROMIS, disease-specific tools) > Search first: EQ-5D database, SF-36, PROMIS, PubMed
  • Disease Course:
  • Complications (secondary problems: infections, organ failure, etc.) > Search first: ICD codes, disease registries, clinical databases, PubMed
  • Recovery potential (likelihood and extent of recovery, with vs without treatment) > Search first: Natural history studies, rehabilitation databases, PubMed
  • Prediction:
  • Prognostic factors (age, disease severity, biomarkers, treatment response) > Search first: Prognostic models databases, clinical calculators, PubMed
  • Prognostic biomarkers (molecular markers predicting disease course) > Search first: FDA Biomarker database, PubMed, cancer prognostic databases

12. Treatment

  • Pharmacotherapy:
  • Pharmacological treatments (drug names, drug classes, mechanisms of action) > Search first: DrugBank, RxNorm, ATC classification, DailyMed, FDA databases
  • Pharmacogenomics (how genetic variants affect drug metabolism, efficacy, toxicity) > Search first: PharmGKB, CPIC (Clinical Pharmacogenetics), FDA Table of PGx Biomarkers
  • Advanced Therapeutics:
  • Gene therapy (viral vectors, CRISPR, gene replacement, gene editing) > Search first: ClinicalTrials.gov, FDA gene therapy database, ASGCT resources
  • Cell therapy (stem cell transplant, CAR-T, cellular therapeutics) > Search first: ClinicalTrials.gov, FDA cell therapy database, FACT standards
  • RNA-based therapies (ASOs, siRNA, mRNA therapies) > Search first: ClinicalTrials.gov, FDA approvals, PubMed
  • Targeted therapies (treatments directed at specific molecular targets) > Search first: My Cancer Genome, OncoKB, ClinicalTrials.gov, FDA approvals
  • Immunotherapies (checkpoint inhibitors, monoclonal antibodies) > Search first: Cancer Immunotherapy Database, FDA approvals, ClinicalTrials.gov
  • Surgical and Interventional:
  • Surgical interventions (types of surgery, timing, outcomes) > Search first: CPT codes, surgical registries, clinical guidelines, PubMed
  • Supportive and Rehabilitative:
  • Supportive care (symptom management, pain control, nutrition) > Search first: Clinical guidelines, Cochrane Library, PubMed
  • Rehabilitation (physical therapy, occupational therapy, speech therapy) > Search first: Rehabilitation medicine databases, clinical guidelines, PubMed
  • Experimental:
  • Experimental treatments in clinical trials (with NCT identifiers if available) > Search first: ClinicalTrials.gov, EU Clinical Trials Register, WHO ICTRP
  • Treatment Outcomes:
  • Treatment response rates > Search first: Clinical trial databases, FDA reviews, systematic reviews, PubMed
  • Side effects and adverse events > Search first: FDA Adverse Event Reporting System (FAERS), MedWatch, PubMed
  • Treatment Strategy:
  • Treatment algorithms (clinical pathways, decision trees) > Search first: Clinical practice guidelines, NCCN Guidelines, UpToDate
  • Combination therapies > Search first: ClinicalTrials.gov, treatment guidelines, PubMed
  • Personalized medicine approaches (genotype-guided treatment) > Search first: My Cancer Genome, CIViC, PharmGKB, precision medicine databases

For each treatment, suggest MAXO (Medical Action Ontology) terms where applicable.

13. Prevention

  • Prevention Levels:
  • Primary prevention (preventing disease occurrence: vaccination, risk factor modification) > Search first: CDC, WHO, USPSTF recommendations, Cochrane Library
  • Secondary prevention (early detection and treatment: screening programs, early intervention) > Search first: USPSTF, CDC screening guidelines, WHO
  • Tertiary prevention (preventing complications in those with disease) > Search first: Clinical guidelines, disease management protocols, PubMed
  • Immunization: Vaccine strategies (if applicable)

    Search first: CDC vaccine schedules, WHO immunization, FDA vaccine database

  • Screening and Early Detection:
  • Screening programs (population-based: newborn screening, cancer screening) > Search first: CDC screening programs, USPSTF, cancer screening databases
  • Genetic screening (carrier screening, preimplantation genetic diagnosis, prenatal testing) > Search first: ACMG recommendations, ACOG guidelines, GTR
  • Risk stratification (identifying high-risk individuals for targeted prevention) > Search first: Risk prediction models, clinical calculators, PubMed
  • Behavioral Interventions: Lifestyle modifications to reduce risk

    Search first: CDC, WHO, behavioral intervention databases, Cochrane Library

  • Counseling: Genetic counseling (risk assessment, family planning guidance)

    Search first: NSGC resources, ACMG guidelines, GeneReviews

  • Public Health:
  • Public health interventions (sanitation, vector control, health education) > Search first: CDC, WHO, public health databases, PubMed
  • Environmental interventions (reducing environmental risk factors) > Search first: EPA databases, WHO environmental health, PubMed
  • Prophylaxis: Preventive medications or procedures

    Search first: Clinical guidelines, FDA approvals, PubMed

14. Other Species / Natural Disease

  • Taxonomy: Species affected (with NCBI Taxon identifiers)

    Search first: NCBI Taxonomy

  • Breed: Specific breeds affected (with VBO identifiers if applicable)

    Search first: VBO (Vertebrate Breed Ontology)

  • Gene: Orthologous genes in other species (with NCBI Gene IDs)

    Search first: NCBI Gene

  • Natural Disease:
  • Naturally occurring disease in other species (companion animals, wildlife) > Search first: OMIA (Online Mendelian Inheritance in Animals), VetCompass, PubMed
  • Veterinary relevance and importance in animal health > Search first: OMIA, veterinary databases, PubMed
  • Comparative Biology:
  • Comparative pathology (similarities and differences across species) > Search first: OMIA, comparative pathology databases, PubMed
  • Evolutionary conservation of disease mechanisms > Search first: HomoloGene, OrthoMCL, Alliance of Genome Resources
  • Transmission (if applicable):
  • Zoonotic potential > Search first: CDC zoonotic diseases, WHO zoonoses, GIDEON
  • Cross-species susceptibility > Search first: NCBI Taxonomy, veterinary databases, PubMed

15. Model Organisms

  • Model Types:
  • Model organism type (mammalian, invertebrate, cellular, in vitro) > Search first: Alliance of Genome Resources, model organism databases
  • Specific model systems (mouse, rat, zebrafish, Drosophila, C. elegans, yeast, cell lines, organoids, iPSCs) > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, SGD, ATCC, Cellosaurus
  • Induced models (drug treatment, surgical intervention, environmental manipulation) > Search first: MGI, model organism databases, PubMed
  • Genetic Models:
  • Types available (knockout, knock-in, transgenic, conditional, humanized) > Search first: MGI, IMPC, KOMP, EuMMCR, IMSR
  • Model Characteristics:
  • Phenotype recapitulation (how well model reproduces human disease features) > Search first: Model organism databases, comparative studies, PubMed
  • Model limitations (aspects of human disease not captured) > Search first: Model organism databases, PubMed, review articles
  • Applications:
  • Research applications (what aspects of disease can be studied) > Search first: Model organism databases, PubMed
  • Resources:
  • Model databases > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, IMSR, EMMA, MMRRC

Citation Requirements

  • Cite primary literature (PMID preferred) for all mechanistic and clinical claims
  • Prioritize recent reviews and landmark papers
  • Include direct quotes from abstracts where possible to support key statements
  • Distinguish evidence source types: human clinical, model organism, in vitro, computational

Output Format

Structure your response as a comprehensive narrative organized by the sections above. For each section, provide: - Factual content with specific details (numbers, percentages, gene names, variant nomenclature) - Ontology term suggestions (HPO, GO, CL, UBERON, CHEBI, MAXO, MONDO) where applicable - Evidence citations with PMIDs - Direct quotes from abstracts to support key claims - Clear indication when information is not available or not applicable for this disease

This report will be used to populate a disease knowledge base entry with: - Pathophysiology descriptions with causal chains - Gene/protein annotations (HGNC, GO terms) - Phenotype associations (HP terms) with frequencies - Cell type involvement (CL terms) - Anatomical locations (UBERON terms) - Chemical entities (CHEBI terms) - Treatment annotations (MAXO terms) - Evidence items with PMIDs and exact abstract quotes - Epidemiology, prognosis, diagnostic, and prevention information - Animal model descriptions with phenotype recapitulation details

Meningioma (Neoplastic) — Disease Characteristics Research Report (2023–2024 emphasis)

Executive summary

Meningiomas are the most common primary intracranial tumors in adults and comprise the majority of non‑malignant primary brain/CNS tumors in US registry data. Contemporary disease management is increasingly driven by integrated histology plus molecular profiling, particularly DNA‑methylation classes and specific high‑risk alterations (TERT promoter mutation; CDKN2A/B loss) that are now incorporated into WHO grading. Standard-of-care remains local therapy (surgery ± radiotherapy), while no FDA‑approved systemic therapy exists for aggressive/recurrent disease; multiple molecularly stratified targeted, immunotherapy, and radionuclide trials are ongoing. (wang2024meningiomainternationalconsortium pages 4-4, deng2022moleculardiagnosisand pages 1-2, lotsch2024themolecularand pages 1-2)

Domain Key points Quantitative highlights Source / URL / date Evidence
Definition & classification Meningioma is the most common primary intracranial tumor in adults and a single WHO CNS tumor type with 15 histologic subtypes. WHO CNS5 (2021) grades meningiomas as CNS WHO grades 1–3; grade 3 can be assigned by molecular criteria even without classic anaplastic histology when TERT promoter mutation or CDKN2A/B homozygous deletion is present. Most cases are grade 1; grade 2–3 comprise the more aggressive subset. ~80% grade 1; up to ~20% grade 2/3. WHO 2021 added TERTp mutation and CDKN2A/B homozygous deletion as grade 3 criteria. Louis et al., Neuro-Oncology Jun 2021, https://doi.org/10.1093/neuonc/noab106; Deng et al., Chinese Medical Journal Sep 2022, https://doi.org/10.1097/cm9.0000000000002391; Torp et al., Acta Neurochirurgica Jul 2022, https://doi.org/10.1007/s00701-022-05301-y (deng2022moleculardiagnosisand pages 1-2, torp2022thewho2021 pages 7-9)
Epidemiology Recent US registry data show meningioma dominates the nonmalignant CNS tumor burden. Figure-based CBTRUS/consensus data indicate higher incidence in females and in Black individuals relative to several comparison groups. Risk rises with age. Nonmalignant distribution: meningiomas 56.2% of nonmalignant primary brain tumors. All primary brain tumors: 5-year total 453,623; annual average 90,725. Nonmalignant tumors: 5-year total 326,894; annual average 65,379. Earlier CBTRUS series: meningioma 39.0% of all tumors and 54.5% of nonmalignant tumors. Price et al., CBTRUS report, Neuro-Oncology Oct 2024, https://doi.org/10.1093/neuonc/noae145; Wang et al., Neuro-Oncology May 2024, https://doi.org/10.1093/neuonc/noae082; Wang et al., Adv Exp Med Biol Jan 2023, https://doi.org/10.1007/978-3-031-29750-2_11 (wang2024meningiomainternationalconsortium pages 4-4, wang2024meningiomainternationalconsortium media 5c0c850b, wang2023genomiclandscapeof pages 1-2)
Molecular drivers Canonical split is NF2-mutant versus non-NF2 meningioma. NF2 alteration is the dominant event in sporadic disease; non-NF2 tumors are enriched for TRAF7, KLF4, AKT1, SMO, PIK3CA, POLR2A and often correlate with skull-base location / specific histologies. NF2 altered in ~40–60% (up to ~60%) of sporadic cases. Approximate non-NF2 frequencies: TRAF7 20–25%, KLF4 10–15%, AKT1 ~10%, SMO 1–5%, PIK3CA ~5%. Wang et al., J Neuro-Oncol Jan 2023, https://doi.org/10.1007/s11060-023-04253-2; Hsieh et al., Cancer May 2024, https://doi.org/10.1002/cncr.35279; Lotsch et al., IJMS Sep 2024, https://doi.org/10.3390/ijms25179631 (wang2023themultiomiclandscape pages 1-2, hsieh2024evolvingconceptsin pages 1-3, lotsch2024themolecularand pages 2-4)
Molecular subgroups & CNVs Contemporary methylation/integrated classifications identify biologically meaningful subgroups. One widely used framework recognizes Immunogenic, NF2-wild-type, Hypermetabolic, and Proliferative groups; aggressive groups carry greater chromosomal instability. Recurrent CNVs include losses of 22q, 1p, 14q and, in more aggressive tumors, additional losses such as 10, 18. Hypermetabolic/Proliferative tumors show higher CNV burden with losses including 1p, 10, 14, 18, 22q. WHO grade 2/3 tumors have more genomic disruption than grade 1. Wang et al., Nature Medicine Aug 2024, https://doi.org/10.1038/s41591-024-03167-4; Lotsch et al., IJMS Sep 2024, https://doi.org/10.3390/ijms25179631; Marastoni & Barresi, Cancers May 2023, https://doi.org/10.3390/cancers15112945 (wang2024molecularclassificationto pages 1-2, lotsch2024themolecularand pages 2-4, marastoni2023meningiomagradingbeyond pages 1-2)
Prognostic biomarkers: TERTp TERT promoter mutation is a high-risk biomarker enriched in higher-grade/aggressive meningiomas and now incorporated into WHO grade 3 criteria. TERTp mutation frequency reported as 4.7% in WHO 1, 7.9% in WHO 2, 15.4% in WHO 3; associated PFS 14 months vs 101 months for TERTp-mutant vs wild-type. Lotsch et al., IJMS Sep 2024, https://doi.org/10.3390/ijms25179631 (lotsch2024themolecularand pages 2-4)
Prognostic biomarkers: CDKN2A/B CDKN2A/B deletion is strongly associated with progression and poor PFS; homozygous loss is a WHO grade 3 criterion, while even heterozygous loss may be adverse. Overall prevalence of homozygous CDKN2A/B deletion 4.9%. Median PFS: 180.0 months (WT) vs 26.1 months (heterozygous deletion) vs 11.0 months (homozygous deletion). Meta-analysis HR for progression: 5.5 (heterozygous) and 8.4 (homozygous). Wach et al., Acta Neuropathol Commun Nov 2023, https://doi.org/10.1186/s40478-023-01690-y; Lotsch et al., IJMS Sep 2024, https://doi.org/10.3390/ijms25179631 (lotsch2024themolecularand pages 2-4, bhala2021incidenceofbenign pages 3-3)
Prognostic biomarkers: grade 2 integrated risk DNA methylation/copy-number/TERTp-integrated risk stratification refines recurrence prediction in histologic grade 2 disease beyond morphology alone. In a grade 2 cohort (n=100), local control 84.3% at 2 y, 68.5% at 4 y, 50.8% at 6 y; integrated risk HR for local recurrence: 9.91 (intermediate) and 7.29 (high) vs low-risk; GTR HR 0.19 for local progression. Ehret et al., Acta Neuropathol Commun May 2024, https://doi.org/10.1186/s40478-024-01739-6 (marastoni2023meningiomagradingbeyond pages 1-2)
Prognostic biomarkers: surgery / Simpson Extent of resection remains a major real-world prognostic factor across molecular groups. Dural margin treatment matters. Nature Medicine 2024 matched analysis: subtotal resection vs GTR associated with worse PFS HR 2.02; Simpson grade 3 vs Simpson 1/2 associated with shorter time to recurrence HR 1.64. Wang et al., Nature Medicine Aug 2024, https://doi.org/10.1038/s41591-024-03167-4 (wang2024molecularclassificationto pages 1-2)
Diagnostic / phenotypic notes MRI is the preferred imaging modality; meningiomas usually show avid gadolinium enhancement and often a dural tail. CT helps detect calcification and skull-base bone changes. Many present with mass effect symptoms or seizures; edema can be substantial in selected subtypes. Dural tail reported in up to 72%; calcification on non-contrast CT in up to 25%; perilesional edema in roughly 50%. Wang et al., Neuro-Oncology May 2024, https://doi.org/10.1093/neuonc/noae082; Pacult et al., Cancers May 2024, https://doi.org/10.3390/cancers16111978 (wang2024meningiomainternationalconsortium pages 14-14, pacult2024surgicalmanagementof pages 1-2)
Systemic therapy status Standard management remains surgery ± radiotherapy. No systemic therapy is established as routine standard; no FDA-approved systemic therapy is available for aggressive meningioma. Molecularly guided therapy, immunotherapy, and radionuclide therapy are active investigation areas. EANO: no target achieved ESCAT I; systemic/radioligand approaches remain investigational. Reifenberger et al., EANO guideline 2024; Lotsch et al., IJMS Sep 2024, https://doi.org/10.3390/ijms25179631; Hsieh et al., Cancer May 2024, https://doi.org/10.1002/cncr.35279 (reifenberger2024eanoguidelineon pages 4-5, lotsch2024themolecularand pages 1-2, hsieh2024evolvingconceptsin pages 10-11, reifenberger2024eanoguidelineon pages 3-4, reifenberger2024eanoguidelineon pages 20-21)
Example trial: Alliance A071401 Molecularly stratified phase II platform for progressive meningioma. Arms are matched to pathway alterations. NCT02523014; arms: vismodegib (SMO/PTCH1), GSK2256098 (FAK; NF2), capivasertib (AKT1/PIK3CA/PTEN), abemaciclib (CDK pathway). Primary endpoint: 6-month PFS. Status: recruiting record with no posted results in retrieved excerpt. ClinicalTrials.gov record (Alliance for Clinical Trials in Oncology), 2015, https://clinicaltrials.gov/study/NCT02523014 (NCT02523014 chunk 1)
Example trial: pembrolizumab Phase II single-arm immunotherapy for recurrent or residual high-grade meningioma. NCT03279692; pembrolizumab q3 weeks; primary endpoint PFS at 6 months; enrollment 26; results first posted 2023-06-06 in registry metadata. ClinicalTrials.gov record (Massachusetts General Hospital), 2017, https://clinicaltrials.gov/study/NCT03279692 (NCT03279692 chunk 1, NCT03279692 chunk 2)
Example trial: nivolumab ± ipilimumab Phase II immunotherapy study in recurrent/progressive meningioma. NCT02648997; nivolumab alone or nivolumab + ipilimumab after RT; actual enrollment 40; primary endpoint 6-month non-progression; secondary endpoints include median PFS, OS, ORR, CTCAE AEs. ClinicalTrials.gov record (Dana-Farber), 2016, https://clinicaltrials.gov/study/NCT02648997 (NCT02648997 chunk 1, NCT02648997 chunk 3)
Example trials: radionuclide therapy SSTR2-targeted theranostics are emerging for recurrent/progressive disease. NCT06326190 (EORTC/LUMEN-1 mentioned in EANO context) and NCT06955169 MOMENTUM-1 compare/assess [177Lu]Lu-DOTATATE in SSTR2-positive recurrent/progressive meningioma; MOMENTUM-1 randomizes against local SOC and uses PFS as primary endpoint. EANO guideline 2024; ClinicalTrials.gov, 2024–2025, https://clinicaltrials.gov/study/NCT06326190 and https://clinicaltrials.gov/study/NCT06955169 (reifenberger2024eanoguidelineon pages 12-12, NCT06955169 chunk 1)
Example trial: combination targeted radionuclide + mTOR inhibitor Combination strategy for refractory higher-grade meningioma. NCT06126588 (ELUMEN); everolimus + 177Lu-DOTATATE in refractory WHO grade 2–3 meningioma; primary endpoint PFS at 7 months; planned enrollment 28. ClinicalTrials.gov record (Central Hospital, Nancy, France), 2024, https://clinicaltrials.gov/study/NCT06126588 (NCT06126588 chunk 1)

Table: This table compacts the most actionable current evidence on meningioma classification, epidemiology, molecular biology, prognosis, and investigational systemic therapies. It is designed as a quick-reference artifact for building a disease knowledge base entry with traceable citations.

1. Disease information

1.1 Definition and overview

Meningiomas arise from the meninges and are typically slow growing, but a clinically important subset behaves aggressively and recurs despite therapy. A 2024 consensus review notes that “Meningiomas are the most common primary intracranial tumors in adults” and that incidence is increasing with aging and increased neuroimaging utilization. (wang2024meningiomainternationalconsortium pages 4-4)

1.2 Classification and grading (current understanding)

The WHO CNS5 (2021) framework treats meningioma as a single tumor type with multiple histologic subtypes and assigns CNS WHO grades 1–3 based on histopathologic features and select molecular criteria. (torp2022thewho2021 pages 7-9, deng2022moleculardiagnosisand pages 1-2)

Key grading points captured in recent sources: - WHO CNS5 recognizes 15 histological subtypes, with grade assignment reflecting subtype and malignancy features. (torp2022thewho2021 pages 7-9) - Chordoid and clear cell meningiomas are assigned grade 2 due to higher recurrence risk; anaplastic meningioma is grade 3. (torp2022thewho2021 pages 7-9) - Importantly, WHO CNS5 integrates molecular markers into grading; a 2022 expert consensus summarizes that tumors “harboring TERT promoter mutation and/or CDKN2A/B homozygous deletion are allotted to WHO grade 3 regardless of histologic anaplasia.” (deng2022moleculardiagnosisand pages 1-2)

Direct abstract quote (grading biomarker integration): The 2023 review on grading beyond histopathology states: “The grading system established by the World Health Organization has recently included pTERT mutations and CDKN2A/B homozygous deletions as criteria for grade 3…” (marastoni2023meningiomagradingbeyond pages 1-2)

1.3 Key identifiers (ontology/terminology)

Within the retrieved evidence for this run, authoritative cross‑ontology identifiers (e.g., MONDO ID, MeSH descriptor code, ICD‑10/ICD‑11 codes, Orphanet) were not directly available as citable text. Consequently, I do not assert specific identifier values without additional retrieval of those controlled‑vocabulary resources.

1.4 Synonyms / alternative names

High‑grade meningiomas are often referred to clinically as “atypical” (WHO grade 2) and “anaplastic/malignant” (WHO grade 3) meningiomas. (pacult2024surgicalmanagementof pages 1-2)

1.5 Evidence source type

The report integrates evidence from: - Aggregated disease-level resources (registry/CBTRUS analyses; consensus reviews). (wang2024meningiomainternationalconsortium pages 4-4, wang2023genomiclandscapeof pages 1-2) - Human clinical cohorts (molecular prognostic cohorts; surgical/radiation outcome cohorts). (wang2024molecularclassificationto pages 1-2, marastoni2023meningiomagradingbeyond pages 1-2) - ClinicalTrials.gov trial records. (NCT02523014 chunk 1, NCT03279692 chunk 1, NCT02648997 chunk 1, NCT06955169 chunk 1, NCT06126588 chunk 1)

2. Etiology

2.1 Causal factors and mechanisms (high-level)

Meningioma development is driven by recurrent somatic alterations that define molecular subgroups and influence anatomic distribution and phenotype (e.g., NF2‑driven versus non‑NF2‑driven disease). (wang2023themultiomiclandscape pages 1-2, torp2022thewho2021 pages 7-9)

2.2 Risk factors

  • Ionizing radiation: A SEER‑focused epidemiology analysis states: “The only currently established environmental risk factor is ionizing radiation.” (bhala2021incidenceofbenign pages 3-3)
  • Age and sex: Risk increases with age and is higher in females for largely non‑malignant lesions; sex differences are also supported by registry‑derived figures in the 2024 consensus epidemiology figure. (bhala2021incidenceofbenign pages 3-3, wang2024meningiomainternationalconsortium media 5c0c850b)
  • Race/ethnicity: Elevated risk among non‑Hispanic Black individuals is noted in recent discussions of US registry patterns and shown in the consensus epidemiology figure panels. (hsieh2024evolvingconceptsin pages 1-3, wang2024meningiomainternationalconsortium media 5c0c850b)

2.3 Protective factors

No protective factors were identified in the retrieved evidence excerpts for this run.

2.4 Gene–environment interactions

Gene–environment interaction evidence was not directly extractable from the retrieved excerpts.

3. Phenotypes

3.1 Common clinical presentation (symptoms/signs)

Meningiomas commonly present with symptoms attributable to mass effect and cortical irritation. - The 2024 consensus review summarizes that many present with “mass-effect symptoms or seizures.” (wang2024meningiomainternationalconsortium pages 14-14)

Suggested HPO terms (symptoms/signs): - Seizures — HP:0001250 (supported by clinical presentation statement) (wang2024meningiomainternationalconsortium pages 14-14) - Headache — HP:0002315 (mass-effect symptom; commonly reported clinically, but explicit frequency not provided in retrieved excerpts) - Focal neurological deficit — HP:0001249 (mass effect; explicit enumeration not provided in retrieved excerpts)

3.2 Imaging phenotype (diagnostic radiology)

MRI is the preferred modality. - The consensus review reports that meningiomas “avidly enhance with gadolinium” and a dural tail is “reported in up to 72%.” (wang2024meningiomainternationalconsortium pages 14-14) - Non‑contrast CT can show calcification “in up to 25%,” and “roughly 50% may have some perilesional edema.” (wang2024meningiomainternationalconsortium pages 14-14)

Suggested HPO terms (imaging‑linked): - Intracranial mass — HP:0002175 - Cerebral edema — HP:0100749 (perilesional edema) (wang2024meningiomainternationalconsortium pages 14-14) - Intracranial calcification — HP:0002514 (CT calcification) (wang2024meningiomainternationalconsortium pages 14-14)

3.3 Pathology phenotype (histology)

High‑grade meningiomas demonstrate higher mitotic activity and brain invasion. - A 2024 surgical review defines high‑grade meningiomas (WHO grade 2/3) and notes microscopic “high mitotic rates” and macroscopic “brain invasion.” (pacult2024surgicalmanagementof pages 1-2)

Suggested HPO terms (pathology‑linked): - Neoplasm invasiveness (brain invasion) — HP:0032687 (conceptually aligned; term selection may require refinement)

3.4 Frequency/severity/progression

  • Many benign lesions may show “stagnancy or a mean growth of 1–1.5 mm/year” in natural history observations of benign tumors. (hsieh2024evolvingconceptsin pages 1-3)
  • Aggressive subtypes can have poor outcomes; a 2023 review notes a substantial clinically aggressive subset and that aggressive tumors often have “5-year progression free survival (PFS) probability less than 50%.” (wang2023themultiomiclandscape pages 1-2)

Quality-of-life metrics were not directly extractable from retrieved excerpts.

4. Genetic / molecular information

4.1 Recurrent driver genes (somatic)

A contemporary molecular view divides meningiomas into NF2-altered and non‑NF2 tumors. - NF2 alterations are “the most common genetic abnormality… found in up to 60% of sporadic cases.” (wang2023themultiomiclandscape pages 1-2) - Non‑NF2 recurrent drivers include TRAF7, KLF4, AKT1, SMO, PIK3CA, and POLR2A, with approximate frequencies summarized in a 2023 multi‑omics review. (wang2023themultiomiclandscape pages 1-2)

4.2 Germline syndromes and lineage-defining genes

  • WHO CNS5 updates and genomic-era management reviews highlight lineage‑defining alterations in some histologic entities (e.g., SMARCE1 in clear cell; BAP1 in rhabdoid meningioma). (hsieh2024evolvingconceptsin pages 1-3, lotsch2024themolecularand pages 2-4)

4.3 Chromosomal abnormalities / copy number variation (CNVs)

CNVs are central to prognosis and correlate with grade. - Common alterations include losses of “22q, 1p and 14q,” with higher genomic disruption in WHO grade 2–3 tumors. (lotsch2024themolecularand pages 2-4) - In a large outcomes study, aggressive molecular groups (Hypermetabolic/Proliferative) showed higher CNV burden including losses of “1p, 10, 14, 18 and 22q.” (wang2024molecularclassificationto pages 1-2)

4.4 Epigenetics and DNA methylation

DNA‑methylation profiling is increasingly used to refine diagnosis and risk. - A 2024 brief review advocates for methylation profiling in contemporary meningioma management. (wang2023themultiomiclandscape pages 1-2) - A 2023 implementation paper describes integrated risk prediction combining histology, methylation family, and CNVs, emphasizing practical deployment issues. (wang2023themultiomiclandscape pages 1-2)

4.5 High-risk grading biomarkers (WHO-integrated)

  • TERT promoter (TERTp) mutations: Enriched in higher grade and associated with adverse PFS; one 2024 review reports PFS “14 months vs. 101 months” for TERTp mutant versus wild type. (lotsch2024themolecularand pages 2-4)
  • CDKN2A/B deletions: A 2023 individual‑patient‑data meta‑analysis reports median PFS 180.0 months (WT) versus 26.1 months (heterozygous deletion) versus 11.0 months (homozygous deletion), with strong hazard ratios for progression. (bhala2021incidenceofbenign pages 3-3)

5. Mechanism / pathophysiology

5.1 Upstream drivers to downstream clinical behavior (causal chain)

1) Initiating genomic/epigenomic alterations (e.g., NF2 loss; pathway-specific non‑NF2 driver mutations; chromosomal instability) drive 2) transcriptional programs (hypermetabolic or proliferative states) and 3) invasive/recurrence phenotypes (brain invasion, rapid progression), yielding 4) clinical endpoints of recurrence and treatment resistance. (wang2023themultiomiclandscape pages 1-2, wang2024molecularclassificationto pages 1-2, pacult2024surgicalmanagementof pages 1-2)

5.2 Key pathways (examples with current emphasis)

Genomics-era management reviews link NF2 biology to signaling programs such as Hippo/YAP and PI3K/AKT/mTOR signaling. (hsieh2024evolvingconceptsin pages 1-3)

Suggested GO Biological Process terms (examples): - Cell cycle process — GO:0022402 (proliferative subgroup biology) (wang2024molecularclassificationto pages 1-2) - Regulation of cell proliferation — GO:0042127 (general)

5.3 Immune microenvironment

Immune‑focused meningioma reviews emphasize immunophenotyping and motivate checkpoint blockade trials, reflecting immune involvement in aggressive subtypes. (lotsch2024themolecularand pages 1-2)

Suggested CL (Cell Ontology) terms (examples): - T cell — CL:0000084 (checkpoint blockade rationale) - Macrophage — CL:0000235 (tumor-associated immune infiltrates; referenced in model discussions) (wang2024meningiomainternationalconsortium pages 26-27)

6. Anatomical structures affected

6.1 Organ/tissue level

  • Primary site: intracranial meninges with frequent dural attachment; also spinal meningiomas occur and are managed using analogous principles. (wang2024meningiomainternationalconsortium pages 25-26)

Suggested UBERON terms (examples): - Meninges — UBERON:0000930 - Dura mater — UBERON:0003129 - Spinal cord meninges — (site; specific UBERON mapping may require further lookup)

7. Temporal development

  • Typical onset is adult; pediatric meningiomas are rare and show distinct distributions and higher grade proportions. (wang2024meningiomainternationalconsortium pages 25-26)
  • Disease course varies from indolent growth (mean 1–1.5 mm/year in benign natural history observations) to aggressive recurrence/progression in higher-grade/molecularly high-risk disease. (hsieh2024evolvingconceptsin pages 1-3, wang2023themultiomiclandscape pages 1-2)

8. Inheritance and population

8.1 Epidemiology (recent statistics; emphasis on 2024 sources)

  • CBTRUS-linked consensus figure indicates that among US non‑malignant primary brain tumors, meningiomas account for 56.2% (distribution panel). (wang2024meningiomainternationalconsortium media 5c0c850b)
  • The same figure provides registry totals for all primary brain tumors (2017–2021) and nonmalignant tumors: 5-year total 453,623 (annual average 90,725) and nonmalignant 5-year total 326,894 (annual average 65,379). (wang2024meningiomainternationalconsortium pages 4-4)
  • Sex and race/ethnicity incidence differences are shown in the figure panels (higher in females; higher in Black individuals), supporting recognized demographic disparities. (wang2024meningiomainternationalconsortium media 5c0c850b)

8.2 Special populations

  • Radiation-induced meningiomas are described as rare (1–2%) and more aggressive, with high cytogenetic burden and frequent 1p loss (>50%). (wang2024meningiomainternationalconsortium pages 25-26)
  • Pediatric meningiomas comprise a small fraction of pediatric brain tumors (2.2–3.6%) and have higher spinal/intraventricular frequencies and more grade 2–3 histologies; NF2 alterations are common in pediatric cases. (wang2024meningiomainternationalconsortium pages 25-26)

9. Diagnostics

9.1 Imaging

  • MRI is preferred; strong gadolinium enhancement is typical; dural tail is commonly observed. (wang2024meningiomainternationalconsortium pages 14-14)
  • CT is helpful for calcification and skull-base/bony assessment. (wang2024meningiomainternationalconsortium pages 14-14, pacult2024surgicalmanagementof pages 1-2)

9.2 Histopathology and grading

  • High-grade tumors show increased mitotic activity and brain invasion; grade 2 criteria include 4–19 mitoses per 10 high‑power fields and/or brain invasion in surgical pathology discussions. (pacult2024surgicalmanagementof pages 2-4)

9.3 Molecular diagnostics

  • DNA methylation profiling and sequencing refine classification and risk stratification but may face access/reimbursement barriers in practice. (wang2024meningiomainternationalconsortium pages 14-14)

9.4 Differential diagnosis (not exhaustively captured)

Liquid biopsy and methylation profiling are described as potentially differentiating meningiomas from imaging mimics, but require external validation. (wang2024meningiomainternationalconsortium pages 14-14)

10. Outcome / prognosis

10.1 Prognostic factors (quantitative highlights)

  • Molecular high-risk markers:
  • TERTp mutations: adverse PFS (14 vs 101 months). (lotsch2024themolecularand pages 2-4)
  • CDKN2A/B deletions: strong PFS impact across deletion states (median PFS 180.0 vs 26.1 vs 11.0 months). (bhala2021incidenceofbenign pages 3-3)
  • Extent of resection:
  • A 2024 Nature Medicine outcomes study found subtotal resection associated with worse PFS (HR 2.02) and Simpson grade 3 with shorter time to recurrence versus Simpson grade 1/2 (HR 1.64). (wang2024molecularclassificationto pages 1-2)
  • Integrated molecular risk for grade 2:
  • In a grade 2 cohort, local control rates were 84.3% (2y), 68.5% (4y), 50.8% (6y); integrated risk group was strongly associated with recurrence (HR 9.91 intermediate; HR 7.29 high vs low), and gross total resection decreased progression risk (HR 0.19). (marastoni2023meningiomagradingbeyond pages 1-2)

10.2 Survival by grade

A 2024 molecular/immunologic review reports grade-associated survival differences (reported survival 12.5 years for grade 1; 6.9 years for grade 2; 2.4 years for grade 3). (lotsch2024themolecularand pages 2-4)

11. Treatment

11.1 Standard-of-care (real-world implementation)

  • Surgery is the cornerstone; maximal safe resection and dural margin management reduce recurrence risk. (pacult2024surgicalmanagementof pages 1-2, wang2024molecularclassificationto pages 1-2)
  • Radiotherapy (adjuvant or definitive) is used in selected settings (particularly high-grade or residual disease), but optimal use remains an area of clinical equipoise in some subgroups and special populations. (marastoni2023meningiomagradingbeyond pages 1-2, wang2024meningiomainternationalconsortium pages 26-27)

11.2 Systemic therapy: current status and expert consensus

Multiple authoritative reviews and guidelines converge on the lack of an established systemic standard: - “Despite intensive research, no systemic treatment options are yet available in the clinic for these challenging tumors…” (review statement). (lotsch2024themolecularand pages 1-2) - EANO guideline: “other treatment options including various systemic therapies and targeted radionuclide therapy have been investigated, but none are established as management standard,” and “no approved targeted treatments are available for this tumor type.” (reifenberger2024eanoguidelineon pages 4-5) - EANO guideline also states: “So far, sufficient data from prospective clinical trials are missing to justify clear recommendations for molecularly targeted therapy in routine practice.” (reifenberger2024eanoguidelineon pages 20-21)

11.3 Recent developments (2023–2024 focus): clinical trials and precision approaches

Key interventional trials retrieved from ClinicalTrials.gov include: - Alliance A071401 (NCT02523014): mutation‑directed arms with vismodegib (SMO/PTCH1), FAK inhibitor GSK2256098 (NF2), capivasertib (AKT1/PIK3CA/PTEN), abemaciclib (CDK pathway). Primary endpoint includes 6‑month PFS. (NCT02523014 chunk 1) - Pembrolizumab (NCT03279692) in recurrent/residual high‑grade meningioma: primary endpoint PFS at 6 months; ClinicalTrials.gov indicates results posted (metadata). (NCT03279692 chunk 1) - Nivolumab ± ipilimumab (NCT02648997): primary endpoint is the number of participants without progression at 6 months; includes a combined regimen after radiotherapy. (NCT02648997 chunk 1) - Radionuclide therapy / theranostics: - EANO notes SSTR2 as an actionable target and identifies a randomized trial effort for [177Lu]Lu‑DOTATATE (LUMEN‑1; NCT06326190 mentioned in guideline context) but emphasizes lack of conclusive controlled trial data to date. (reifenberger2024eanoguidelineon pages 12-12) - MOMENTUM‑1 (NCT06955169): randomized phase 2 comparing [177Lu]Lu‑DOTATATE versus local standard-of-care systemic options, requiring positive [68Ga]Ga‑DOTATATE PET uptake; primary endpoint PFS. (NCT06955169 chunk 1) - ELUMEN (NCT06126588): everolimus + 177Lu‑DOTATATE PRRT in refractory WHO grade 2–3 meningioma; primary endpoint PFS at 7 months. (NCT06126588 chunk 1)

Suggested MAXO terms (examples): - Surgical resection — MAXO:0000004 (conceptual) - Radiotherapy — MAXO:0000014 (conceptual) - Immune checkpoint inhibitor therapy — MAXO term requires lookup; supported as clinical‑trial intervention (NCT03279692 chunk 1, NCT02648997 chunk 1) - Peptide receptor radionuclide therapy (PRRT) — MAXO term requires lookup; supported as trial intervention (NCT06955169 chunk 1, NCT06126588 chunk 1)

12. Prevention

12.1 Primary prevention

No established primary prevention strategy exists for sporadic meningioma in the retrieved excerpts, aside from general avoidance/minimization of unnecessary ionizing radiation exposure (the only established environmental risk factor in the cited epidemiologic analysis). (bhala2021incidenceofbenign pages 3-3)

12.2 Secondary prevention / screening

No population screening paradigm was identified in retrieved excerpts.

12.3 Tertiary prevention

Recurrence risk mitigation relies on maximal safe resection, appropriate adjuvant radiotherapy selection, and emerging molecular risk stratification (e.g., integrated methylation/CNV/biomarker scoring). (wang2024molecularclassificationto pages 1-2, marastoni2023meningiomagradingbeyond pages 1-2)

13. Other species / natural disease

No citable evidence on naturally occurring meningioma in non-human species was retrieved in the excerpts used for this run.

14. Model organisms

A 2024 review on genetics/classification/mouse modeling was retrieved in search results, but model‑organism details were not present in the evidence excerpts used here; thus, I do not provide specific model organism assertions without additional excerpt retrieval. (wang2023themultiomiclandscape pages 1-2)

Figures (registry epidemiology)

The CBTRUS-derived epidemiology visual panels (distribution and incidence by sex/race/grade) were retrieved from the 2024 consensus review figure; these panels support the numeric nonmalignant distribution (meningioma 56.2%) and qualitative sex/race incidence disparities discussed above. (wang2024meningiomainternationalconsortium media 5c0c850b, wang2024meningiomainternationalconsortium media cc65ec92, wang2024meningiomainternationalconsortium media 5f52723b)

Notes on evidence gaps and how they affect this knowledge-base entry

  • Ontology identifiers (MONDO/MeSH/ICD/Orphanet) were not directly retrievable as citable text in the current run; they should be populated from the respective controlled‑vocabulary resources and then cited.
  • Protective factors, gene–environment interactions, and cross‑species disease were not supported by extracted evidence in this run.
  • Model organisms likely exist (as suggested by retrieved review titles), but detailed, citable model descriptions were not extracted in the included evidence snippets.

References

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  23. (NCT02648997 chunk 3): David Reardon, MD. An Open-Label Phase II Study of Nivolumab or Nivolumab/Ipilimumab in Adult Participants With Progessive/ Recurrent Meningioma. Dana-Farber Cancer Institute. 2016. ClinicalTrials.gov Identifier: NCT02648997

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  25. (NCT06955169 chunk 1): Comparing the Radiopharmaceutical Drug, [177Lu]Lu-DOTATATE, to Standard of Care Treatment for Patients With Meningioma That Has Come Back After Prior Treatment. RTOG Foundation, Inc.. 2025. ClinicalTrials.gov Identifier: NCT06955169

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  27. (wang2024meningiomainternationalconsortium pages 26-27): Justin Z Wang, Alexander P Landry, David R Raleigh, Felix Sahm, Kyle M Walsh, Roland Goldbrunner, Leeor S Yefet, Jörg C Tonn, Chloe Gui, Quinn T Ostrom, Jill Barnholtz-Sloan, Arie Perry, Yosef Ellenbogen, C Oliver Hanemann, Gerhard Jungwirth, Michael D Jenkinson, Ghazaleh Tabatabai, Tiit I Mathiesen, Michael W McDermott, Marcos Tatagiba, Christian la Fougère, Sybren L N Maas, Norbert Galldiks, Nathalie L Albert, Priscilla K Brastianos, Felix Ehret, Giuseppe Minniti, Katrin Lamszus, Franz L Ricklefs, Jens Schittenhelm, Katharine J Drummond, Ian F Dunn, Omar N Pathmanaban, Aaron A Cohen-Gadol, Erik P Sulman, Emeline Tabouret, Emelie Le Rhun, Christian Mawrin, Jennifer Moliterno, Michael Weller, Wenya (Linda) Bi, Andrew Gao, Stephen Yip, Maximilian Niyazi, Kenneth Aldape, Patrick Y Wen, Susan Short, Matthias Preusser, Farshad Nassiri, and Gelareh Zadeh. Meningioma: international consortium on meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients. Neuro-Oncology, 26:1742-1780, May 2024. URL: https://doi.org/10.1093/neuonc/noae082, doi:10.1093/neuonc/noae082. This article has 116 citations and is from a domain leading peer-reviewed journal.

  28. (wang2024meningiomainternationalconsortium pages 25-26): Justin Z Wang, Alexander P Landry, David R Raleigh, Felix Sahm, Kyle M Walsh, Roland Goldbrunner, Leeor S Yefet, Jörg C Tonn, Chloe Gui, Quinn T Ostrom, Jill Barnholtz-Sloan, Arie Perry, Yosef Ellenbogen, C Oliver Hanemann, Gerhard Jungwirth, Michael D Jenkinson, Ghazaleh Tabatabai, Tiit I Mathiesen, Michael W McDermott, Marcos Tatagiba, Christian la Fougère, Sybren L N Maas, Norbert Galldiks, Nathalie L Albert, Priscilla K Brastianos, Felix Ehret, Giuseppe Minniti, Katrin Lamszus, Franz L Ricklefs, Jens Schittenhelm, Katharine J Drummond, Ian F Dunn, Omar N Pathmanaban, Aaron A Cohen-Gadol, Erik P Sulman, Emeline Tabouret, Emelie Le Rhun, Christian Mawrin, Jennifer Moliterno, Michael Weller, Wenya (Linda) Bi, Andrew Gao, Stephen Yip, Maximilian Niyazi, Kenneth Aldape, Patrick Y Wen, Susan Short, Matthias Preusser, Farshad Nassiri, and Gelareh Zadeh. Meningioma: international consortium on meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients. Neuro-Oncology, 26:1742-1780, May 2024. URL: https://doi.org/10.1093/neuonc/noae082, doi:10.1093/neuonc/noae082. This article has 116 citations and is from a domain leading peer-reviewed journal.

  29. (pacult2024surgicalmanagementof pages 2-4): Mark A. Pacult, Colin J. Przybylowski, Shaan M. Raza, and Franco DeMonte. Surgical management of high-grade meningiomas. Cancers, 16:1978, May 2024. URL: https://doi.org/10.3390/cancers16111978, doi:10.3390/cancers16111978. This article has 4 citations.

  30. (wang2024meningiomainternationalconsortium media cc65ec92): Justin Z Wang, Alexander P Landry, David R Raleigh, Felix Sahm, Kyle M Walsh, Roland Goldbrunner, Leeor S Yefet, Jörg C Tonn, Chloe Gui, Quinn T Ostrom, Jill Barnholtz-Sloan, Arie Perry, Yosef Ellenbogen, C Oliver Hanemann, Gerhard Jungwirth, Michael D Jenkinson, Ghazaleh Tabatabai, Tiit I Mathiesen, Michael W McDermott, Marcos Tatagiba, Christian la Fougère, Sybren L N Maas, Norbert Galldiks, Nathalie L Albert, Priscilla K Brastianos, Felix Ehret, Giuseppe Minniti, Katrin Lamszus, Franz L Ricklefs, Jens Schittenhelm, Katharine J Drummond, Ian F Dunn, Omar N Pathmanaban, Aaron A Cohen-Gadol, Erik P Sulman, Emeline Tabouret, Emelie Le Rhun, Christian Mawrin, Jennifer Moliterno, Michael Weller, Wenya (Linda) Bi, Andrew Gao, Stephen Yip, Maximilian Niyazi, Kenneth Aldape, Patrick Y Wen, Susan Short, Matthias Preusser, Farshad Nassiri, and Gelareh Zadeh. Meningioma: international consortium on meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients. Neuro-Oncology, 26:1742-1780, May 2024. URL: https://doi.org/10.1093/neuonc/noae082, doi:10.1093/neuonc/noae082. This article has 116 citations and is from a domain leading peer-reviewed journal.

  31. (wang2024meningiomainternationalconsortium media 5f52723b): Justin Z Wang, Alexander P Landry, David R Raleigh, Felix Sahm, Kyle M Walsh, Roland Goldbrunner, Leeor S Yefet, Jörg C Tonn, Chloe Gui, Quinn T Ostrom, Jill Barnholtz-Sloan, Arie Perry, Yosef Ellenbogen, C Oliver Hanemann, Gerhard Jungwirth, Michael D Jenkinson, Ghazaleh Tabatabai, Tiit I Mathiesen, Michael W McDermott, Marcos Tatagiba, Christian la Fougère, Sybren L N Maas, Norbert Galldiks, Nathalie L Albert, Priscilla K Brastianos, Felix Ehret, Giuseppe Minniti, Katrin Lamszus, Franz L Ricklefs, Jens Schittenhelm, Katharine J Drummond, Ian F Dunn, Omar N Pathmanaban, Aaron A Cohen-Gadol, Erik P Sulman, Emeline Tabouret, Emelie Le Rhun, Christian Mawrin, Jennifer Moliterno, Michael Weller, Wenya (Linda) Bi, Andrew Gao, Stephen Yip, Maximilian Niyazi, Kenneth Aldape, Patrick Y Wen, Susan Short, Matthias Preusser, Farshad Nassiri, and Gelareh Zadeh. Meningioma: international consortium on meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients. Neuro-Oncology, 26:1742-1780, May 2024. URL: https://doi.org/10.1093/neuonc/noae082, doi:10.1093/neuonc/noae082. This article has 116 citations and is from a domain leading peer-reviewed journal.