Pediatric meningioma

Summary

Pediatric meningioma (MONDO:0003057) is a disease with 2 cohort genes and 129 clinical trials. Top therapeutic interventions include lutetium oxodotreotide lu-177, edotreotide gallium ga-68, and tranexamic acid.

At a glance

• Cohort genes: 2
• Clinical trials: 129

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	pediatric meningioma
Mondo ID	MONDO:0003057
DOID	DOID:4593
NCIT	C8264
UMLS	C0280656
MedGen	79156
GARD	0023350
Is cancer (heuristic)	no

Also known as: childhood meningioma · childhood meningioma (disease) · meningioma · meningioma (disease) of childhood · paediatric meningioma (disease) · pediatric meningioma · pediatric meningioma (disease)

Data availability: 38 cell lines.

Disease family

An umbrella term covering 2 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › central nervous system neoplasm › tumor of meninges › meningioma › pediatric meningioma

Related subtypes (36): intraspinal meningioma, intraventricular meningioma, intraorbital meningioma, clear cell meningioma, posterior cranial fossa meningioma, anterior cranial fossa meningioma, skull base meningioma, benign meningioma, secretory meningioma, lymphoplasmacyte-rich meningioma, microcystic meningioma, middle cranial fossa meningioma, rhabdoid meningioma, optic nerve sheath meningioma, lung meningioma, malignant leptomeningeal tumor, jugular foramen meningioma, angiomatous meningioma, psammomatous meningioma, fibrous meningioma, meningothelial meningioma, transitional meningioma, petrous apex meningioma, gasserian ganglion meningioma, skin meningioma, periocular meningioma, pineal region meningioma, parapharyngeal meningioma, radiation-induced meningioma, familial meningioma, grade III meningioma, papillary meningioma, optic tract meningioma, grade II meningioma, intracranial meningioma, supratentorial meningioma

Subtypes (2): pediatric leptomeningeal melanoma, childhood brain meningioma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 1 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
LEPR	Orphanet:179494	Obesity due to leptin receptor gene deficiency

Cohort genes → proteins

2 cohort genes, 2 distinct canonical proteins.

Evidence partition

Subset	Genes
civic_only	2

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
LEPR	HGNC:6554	ENSG00000116678	P48357	Leptin receptor	civic_evidence
PTTG1	HGNC:9690	ENSG00000164611	O95997	Securin	civic_evidence

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
LEPR	Leptin receptor	Receptor for hormone LEP/leptin.
PTTG1	Securin	Regulatory protein, which plays a central role in chromosome stability, in the p53/TP53 pathway, and DNA repair.

Protein-family classification

Druggable: 1 · Difficult: 0 · Unknown: 1 · Druggable fraction: 0.5

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Antibody/Immunoglobulin	1	14.6×	0.135
Other/Unknown	1	0.9×	0.805

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
LEPR	Antibody/Immunoglobulin	yes		Hematopoietin_rcpt_Gp130_CS, Hempt_rcpt_S_F1_CS, FN3_dom
PTTG1	Other/Unknown	no		Securin_separation_inhibitor

Expression context

Cohort genes with no expression data: 0.

2 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	2
unknown	0

Top tissues across cohort

Tissue	Cohort genes
choroid plexus epithelium	1
trabecular bone tissue	1
trigeminal ganglion	1
oocyte	1
secondary oocyte	1
ventricular zone	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
LEPR	272	broad	marker	trabecular bone tissue, choroid plexus epithelium, trigeminal ganglion
PTTG1	246	ubiquitous	marker	oocyte, secondary oocyte, ventricular zone

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
LEPR	2,243
PTTG1	2,225

Structural data

PDB: 2 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
LEPR	P48357	9
PTTG1	O95997	2

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 4. Enrichment computed across 2 evidence-associated genes (2 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Signaling by Leptin	1	519.1×	0.008	LEPR
APC/C:Cdc20 mediated degradation of Securin	1	95.2×	0.016	PTTG1
APC/C:Cdh1 mediated degradation of Cdc20 and other APC/C:Cdh1 targeted proteins in late mitosis/early G1	1	85.2×	0.016	PTTG1
Separation of Sister Chromatids	1	30.4×	0.033	PTTG1

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
multicellular organism development	1	4213.0×	0.003	LEPR
regulation of transport	1	4213.0×	0.003	LEPR
sexual reproduction	1	2808.7×	0.003	LEPR
homologous chromosome segregation	1	1685.2×	0.003	PTTG1
regulation of bone remodeling	1	1404.3×	0.003	LEPR
leptin-mediated signaling pathway	1	1203.7×	0.003	LEPR
response to leptin	1	1203.7×	0.003	LEPR
bone growth	1	1203.7×	0.003	LEPR
regulation of feeding behavior	1	936.2×	0.004	LEPR
energy reserve metabolic process	1	526.6×	0.006	LEPR
glial cell proliferation	1	443.5×	0.006	LEPR
negative regulation of gluconeogenesis	1	401.2×	0.006	LEPR
chromosome organization	1	290.6×	0.008	PTTG1
cell surface receptor signaling pathway via STAT	1	280.9×	0.008	LEPR
glycogen metabolic process	1	263.3×	0.008	LEPR
T cell differentiation	1	191.5×	0.010	LEPR
gluconeogenesis	1	162.0×	0.011	LEPR
energy homeostasis	1	135.9×	0.013	LEPR
negative regulation of autophagy	1	129.6×	0.013	LEPR
phagocytosis	1	120.4×	0.013	LEPR
cholesterol metabolic process	1	98.0×	0.015	LEPR
transport across blood-brain barrier	1	89.6×	0.016	LEPR
positive regulation of cold-induced thermogenesis	1	81.8×	0.016	LEPR
cytokine-mediated signaling pathway	1	65.3×	0.019	LEPR
glucose homeostasis	1	65.3×	0.019	LEPR
cell surface receptor signaling pathway	1	32.0×	0.035	LEPR
DNA repair	1	31.9×	0.035	PTTG1
angiogenesis	1	31.2×	0.035	LEPR
cell division	1	23.1×	0.046	PTTG1
spermatogenesis	1	17.6×	0.058	PTTG1

Therapeutics

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 2

Druggability breadth: 1 of 2 evidence-associated genes (50%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

Symbol	Molecules	Max phase
LEPR	0	0
PTTG1	0	0

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
LEPR	3	Binding:3

Pharmacogenomics

Cohort genes with a PharmGKB record: 2; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

0 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	0
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	1	LEPR
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	1	PTTG1

Undrugged target profiles

2 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
LEPR	3	—
PTTG1	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 129.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	66
PHASE2	31
PHASE1/PHASE2	10
PHASE1	8
EARLY_PHASE1	6
PHASE3	5
PHASE4	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04081701	PHASE4	RECRUITING	68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors.
NCT06377371	PHASE4	RECRUITING	Feasibility of Intraoperative Tracing of Meningioma Using [Cu64]DOTATATE
NCT04386642	PHASE4	UNKNOWN	Tranexamic Acid Reduce Blood Loss in Meningioma Resection
NCT00517959	PHASE3	UNKNOWN	SCRT Versus Conventional RT in Children and Young Adults With Low Grade and Benign Brain Tumors
NCT01655927	PHASE3	UNKNOWN	Efficacy of Tranexamic Acid in Brain Tumor Resections
NCT03015701	PHASE3	COMPLETED	S9005 Mifepristone in Meningioma
NCT03558516	PHASE3	COMPLETED	Magnesium and Intraoperative Blood Loss in Meningioma Surgery
NCT04305470	PHASE3	COMPLETED	Gleolan for Visualization of Newly Diagnosed or Recurrent Meningioma
NCT02523014	PHASE2	RECRUITING	Vismodegib, FAK Inhibitor GSK2256098, Capivasertib, and Abemaciclib in Treating Patients With Progressive Meningiomas
NCT02648997	PHASE2	ACTIVE_NOT_RECRUITING	An Open-Label Phase II Study of Nivolumab or Nivolumab/Ipilimumab in Adult Participants With Progessive/ Recurrent Meningioma
NCT02847559	PHASE2	RECRUITING	Optune Delivered Electric Field Therapy and Bevacizumab in Treating Patients With Recurrent or Progressive Grade 2 or 3 Meningioma
NCT03604978	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab and Multi-fraction Stereotactic Radiosurgery With or Without Ipilimumab in Treating Patients With Recurrent Grade II-III Meningioma
NCT03971461	PHASE2	ACTIVE_NOT_RECRUITING	Phase II Study of 177Lu-DOTATATE Radionuclide in Adults With Progressive or High-risk Meningioma
NCT04082520	PHASE2	RECRUITING	Lutathera for the Treatment of Inoperable, Progressive Meningioma After External Beam Radiation Therapy
NCT04298541	PHASE2	NOT_YET_RECRUITING	Direct Comparison of Ga-68-DOTATATE and Ga-68-DOTATOC
NCT04374305	PHASE2	RECRUITING	Innovative Trial for Understanding the Impact of Targeted Therapies in NF2-Related Schwannomatosis (INTUITT-NF2)
NCT04659811	PHASE2	ACTIVE_NOT_RECRUITING	Stereotactic Radiosurgery and Immunotherapy (Pembrolizumab) for the Treatment of Recurrent Meningioma
NCT04997317	PHASE1/PHASE2	RECRUITING	Treatment of Recurrent or Progressive Meningiomas With the Radiolabelled Somatostatin Antagonist 177Lu-satoreotide
NCT05278208	PHASE1/PHASE2	RECRUITING	Lutathera for Treatment of Recurrent or Progressive High-Grade CNS Tumors
NCT05425004	PHASE2	RECRUITING	Cabozantinib for Patients With Recurrent or Progressive Meningioma
NCT05636618	PHASE1/PHASE2	RECRUITING	Targeted Alpha-Particle Therapy for Advanced Somatostatin Receptor Type 2 (SSTR2) Positive Tumors
NCT05940493	PHASE2	RECRUITING	Abemaciclib in Newly Diagnosed Meningioma Patients
NCT06126588	PHASE2	RECRUITING	Combination of Everolimus and 177Lu-DOTATATE in the Treatment of Grades 2 and 3 Refractory Meningioma: a Phase IIb Clinical Trial
NCT06132685	PHASE2	RECRUITING	Post-Operative Dosing of Dexamethasone in Patients With Brain Tumors After a Craniotomy, PODS Trial
NCT06326190	PHASE2	RECRUITING	177Lu-DOTATATE for Recurrent Meningioma
NCT06607692	PHASE1/PHASE2	RECRUITING	Study in Children and Adolescents of 177Lu-DOTATATE (Lutathera®) Combined With the PARP Inhibitor Olaparib for the Treatment of Recurrent or Relapsed Solid Tumours Expressing Somatostatin Receptor (SSTR) (LuPARPed).
NCT06640582	PHASE1/PHASE2	RECRUITING	TIL Therapy Combined With Pembrolizumab for Advanced Brain Cancer Including Gliomas and Meningiomas
NCT06684795	PHASE2	RECRUITING	FG001 in Subjects with Meningiomas or Presumed Low-Grade Gliomas Scheduled for Neurosurgery
NCT06710249	PHASE2	RECRUITING	Impact of Salovum® and SPC® Flakes on Brain Tumor Induced Edema
NCT06804655	PHASE2	NOT_YET_RECRUITING	Pharmacoscopy for Patients With Refractory Primary Brain Tumors
NCT07150806	PHASE1/PHASE2	RECRUITING	RYZ101 for the Treatment of Progressive or Recurrent Intracranial Meningioma
NCT07428616	PHASE2	RECRUITING	A Study of Zanzalintinib in Participants With Recurrent or Progressive Meningioma
NCT07533942	PHASE2	NOT_YET_RECRUITING	A Study of JZP3507 (ONC206) in Recurrent Grade 2 or 3 Meningioma
NCT00003483	PHASE2	TERMINATED	Antineoplaston Therapy in Treating Patients With Meningioma
NCT00589784	PHASE2	COMPLETED	Phase II Trial of Sunitinib (SU011248) in Patients With Recurrent or Inoperable Meningioma
NCT00706810	PHASE2	COMPLETED	Combination of Hydroxyurea and Verapamil for Refractory Meningiomas
NCT00859040	PHASE2	COMPLETED	Monthly SOM230C for Recurrent or Progressive Meningioma
NCT01117844	PHASE1/PHASE2	COMPLETED	Proton Radiation For Meningiomas and Hemangiopericytomas
NCT01967823	PHASE2	COMPLETED	T Cell Receptor Immunotherapy Targeting NY-ESO-1 for Patients With NY-ESO-1 Expressing Cancer
NCT02831257	PHASE2	COMPLETED	AZD2014 In NF2 Patients With Progressive or Symptomatic Meningiomas

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
LUTETIUM OXODOTREOTIDE LU-177	4	4
EDOTREOTIDE GALLIUM GA-68	4	3
TRANEXAMIC ACID	4	3
AMINO ACIDS	4	2
ABEMACICLIB	4	1
ALPELISIB	4	1
AMINOLEVULINIC ACID HYDROCHLORIDE	4	1
BORTEZOMIB	4	1
BRIGATINIB	4	1
CAPIVASERTIB	4	1
HYDROXYUREA	4	1
MIFEPRISTONE	4	1
NERATINIB	4	1
RETIFANLIMAB	4	1
SELUMETINIB	4	1
SUNITINIB	4	1
VERAPAMIL	4	1
VISMODEGIB	4	1
DORDAVIPRONE	3	1
MAGNESIUM	3	1
ZANZALINTINIB	3	1
VISTUSERTIB	2	2
AR-42	2	1
DEXVERAPAMIL	2	1
ELRAGLUSIB	2	1
LYSINE	2	1
ZIRCONIUM ZR 89 CREFMIRLIMAB BERDOXAM	2	1
CHEMBL3527065	0	2
CHEMBL275117	0	1
CHEMBL4517714	0	1