Meningioma is a primarily meningeal central nervous system tumor with a broad clinical spectrum from lower-grade tumors to aggressive high-grade disease.
Conditions with similar clinical presentations that must be differentiated from Meningioma:
name: Meningioma
creation_date: '2026-03-03T21:32:57Z'
updated_date: '2026-03-03T22:06:09Z'
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 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: 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: 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: 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: []