Childhood oligodendroglioma

Summary

Childhood oligodendroglioma (MONDO:0002540) is a disease with 3 cohort genes and 86 clinical trials. Molecularly, MGMT Underexpression confers sensitivity to Temozolomide in Oligodendroglioma (CIViC Level B); 1 further subtype–drug associations are mapped below. Top therapeutic interventions include temozolomide, lapatinib, and edotreotide gallium ga-68.

At a glance

• Cohort genes: 3
• Clinical trials: 86
• Precision-medicine evidence (CIViC): 2 subtype–drug associations

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	childhood oligodendroglioma
Mondo ID	MONDO:0002540
DOID	DOID:3183
NCIT	C4045
UMLS	C0280475
MedGen	76116
GARD	0023156
Is cancer (heuristic)	no

Also known as: oligodendroglioma · oligodendroglioma of childhood · paediatric oligodendroglioma · pediatric oligodendroglioma

Data availability: 26 cell lines.

Disease family

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › childhood malignant neoplasm › childhood oligodendroglioma

Related subtypes (29): pediatric osteosarcoma, pediatric fibrosarcoma, childhood choroid plexus carcinoma, childhood central nervous system primitive neuroectodermal neoplasm, childhood brain stem neoplasm, pediatric angiosarcoma, pediatric mesenchymal chondrosarcoma, pediatric liposarcoma, pediatric lymphoma, childhood malignant mesenchymoma, pediatric myxoid chondrosarcoma, childhood botryoid rhabdomyosarcoma, pediatric intraocular retinoblastoma, childhood cerebral astrocytoma, childhood epithelioid sarcoma, childhood pleomorphic rhabdomyosarcoma, pediatric infratentorial ependymoma, pediatric supratentorial ependymoma, childhood malignant schwannoma, pediatric extraocular retinoblastoma, childhood leukemia, childhood precursor T-lymphoblastic lymphoma/leukemia, malignant childhood germ cell neoplasm, pleuropulmonary blastoma, pediatric hepatocellular carcinoma, childhood malignant kidney neoplasm, childhood malignant melanoma, extrarenal rhabdoid tumor, pediatric high-grade glioma

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: 29 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
TP53	Orphanet:1333	Familial pancreatic carcinoma
TP53	Orphanet:145	Hereditary breast and/or ovarian cancer syndrome
TP53	Orphanet:1501	Adrenocortical carcinoma
TP53	Orphanet:210159	Adult hepatocellular carcinoma
TP53	Orphanet:251576	Gliosarcoma
TP53	Orphanet:251579	Giant cell glioblastoma
TP53	Orphanet:251899	Choroid plexus carcinoma
TP53	Orphanet:2807	Papilloma of choroid plexus
TP53	Orphanet:293199	Pleomorphic rhabdomyosarcoma
TP53	Orphanet:3318	Essential thrombocythemia
TP53	Orphanet:524	Li-Fraumeni syndrome
TP53	Orphanet:52688	Myelodysplastic syndrome
TP53	Orphanet:585909	B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2)
TP53	Orphanet:667662	Breast implant-associated anaplastic large cell lymphoma
TP53	Orphanet:668	Osteosarcoma
TP53	Orphanet:67038	B-cell chronic lymphocytic leukemia
TP53	Orphanet:70573	Small cell lung cancer
TP53	Orphanet:96253	Cushing disease
TP53	Orphanet:99756	Alveolar rhabdomyosarcoma
TP53	Orphanet:99757	Embryonal rhabdomyosarcoma
IDH1	Orphanet:163634	Maffucci syndrome
IDH1	Orphanet:251576	Gliosarcoma
IDH1	Orphanet:251579	Giant cell glioblastoma
IDH1	Orphanet:296	Ollier disease
IDH1	Orphanet:86845	Acute myeloid leukaemia with myelodysplasia-related features
IDH1	Orphanet:99646	Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
MGMT	Orphanet:251576	Gliosarcoma
MGMT	Orphanet:251579	Giant cell glioblastoma
MGMT	Orphanet:618	Familial melanoma

Cohort genes → proteins

3 cohort genes, 3 distinct canonical proteins.

Evidence partition

Subset	Genes
civic_only	3

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
TP53	HGNC:11998	ENSG00000141510	P04637	Cellular tumor antigen p53	civic_evidence
IDH1	HGNC:5382	ENSG00000138413	O75874	Isocitrate dehydrogenase [NADP] cytoplasmic	civic_evidence
MGMT	HGNC:7059	ENSG00000170430	P16455	Methylated-DNA–protein-cysteine methyltransferase	civic_evidence

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
TP53	Cellular tumor antigen p53	Multifunctional transcription factor that induces cell cycle arrest, DNA repair or apoptosis upon binding to its target DNA sequence.
IDH1	Isocitrate dehydrogenase [NADP] cytoplasmic	Catalyzes the NADP(+)-dependent oxidative decarboxylation of isocitrate (D-threo-isocitrate) to 2-ketoglutarate (2-oxoglutarate), which is required by other enzymes such as the phytanoyl-CoA dioxygenase.
MGMT	Methylated-DNA–protein-cysteine methyltransferase	Involved in the cellular defense against the biological effects of O6-methylguanine (O6-MeG) and O4-methylthymine (O4-MeT) in DNA.

Protein-family classification

Druggable: 2 · Difficult: 1 · Unknown: 0 · Druggable fraction: 0.67

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
Enzyme (other)	2	8.0×	0.039
Transcription factor	1	2.8×	0.321

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
TP53	Transcription factor	no		p53_tumour_suppressor, p53-like_TF_DNA-bd_sf, p53_tetrameristn
IDH1	Enzyme (other)	yes	1.1.1.42	Isocitrate_DH_NADP, IsoCit/isopropylmalate_DH_CS, IsoPropMal-DH-like_dom
MGMT	Enzyme (other)	yes	2.1.1.63	MethylDNA_cys_MeTrfase_AS, MethylG_MeTrfase_N, MethylDNA_cys_MeTrfase_DNA-bd

Expression context

Cohort genes with no expression data: 0.

3 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)	3
unknown	0

Top tissues across cohort

Tissue	Cohort genes
ganglionic eminence	1
tendon of biceps brachii	1
ventricular zone	1
adrenal tissue	1
corpus epididymis	1
jejunal mucosa	1
endometrium epithelium	1
liver	1
right lobe of liver	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
TP53	223	ubiquitous	marker	ventricular zone, ganglionic eminence, tendon of biceps brachii
IDH1	294	ubiquitous	marker	corpus epididymis, jejunal mucosa, adrenal tissue
MGMT	261	ubiquitous	marker	right lobe of liver, liver, endometrium epithelium

Protein interactions among cohort

Intra-cohort edges: 3.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
TP53	22,736
IDH1	5,464
MGMT	2,853

Intra-cohort edges

A	B	Sources
IDH1	MGMT	string_interaction
IDH1	TP53	string_interaction
MGMT	TP53	string_interaction

Structural data

PDB: 3 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
TP53	P04637	313
IDH1	O75874	61
MGMT	P16455	23

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 54. Enrichment computed across 3 evidence-associated genes (3 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 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate	1	3806.7×	0.005	IDH1
MGMT-mediated DNA damage reversal	1	3806.7×	0.005	MGMT
Loss of function of TP53 in cancer due to loss of tetramerization ability	1	3806.7×	0.005	TP53
NADPH regeneration	1	1903.3×	0.006	IDH1
Regulation of TP53 Expression	1	1903.3×	0.006	TP53
NFE2L2 regulating TCA cycle genes	1	1268.9×	0.007	IDH1
Transcriptional activation of cell cycle inhibitor p21	1	951.7×	0.008	TP53
Activation of NOXA and translocation to mitochondria	1	634.4×	0.010	TP53
DNA Damage Reversal	1	543.8×	0.010	MGMT
RUNX3 regulates CDKN1A transcription	1	543.8×	0.010	TP53
PI5P Regulates TP53 Acetylation	1	423.0×	0.011	TP53
Activation of PUMA and translocation to mitochondria	1	380.7×	0.011	TP53
TP53 Regulates Transcription of Caspase Activators and Caspases	1	317.2×	0.011	TP53
TP53 Regulates Transcription of Death Receptors and Ligands	1	317.2×	0.011	TP53
Urea cycle	1	292.8×	0.011	TP53
Regulation of TP53 Activity through Association with Co-factors	1	271.9×	0.011	TP53
TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain	1	253.8×	0.011	TP53
Stabilization of p53	1	253.8×	0.011	TP53
TP53 Regulates Transcription of Genes Involved in G1 Cell Cycle Arrest	1	237.9×	0.011	TP53
Formation of Senescence-Associated Heterochromatin Foci (SAHF)	1	223.9×	0.011	TP53
Zygotic genome activation (ZGA)	1	223.9×	0.011	TP53
Regulation of NF-kappa B signaling	1	211.5×	0.011	TP53
TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest	1	200.3×	0.011	TP53
SUMOylation of transcription factors	1	190.3×	0.011	TP53
TP53 Regulates Transcription of Genes Involved in Cytochrome C Release	1	181.3×	0.011	TP53
Regulation of TP53 Activity through Methylation	1	181.3×	0.011	TP53
TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain	1	173.0×	0.012	TP53
Regulation of TP53 Activity through Acetylation	1	152.3×	0.013	TP53
Pyroptosis	1	141.0×	0.013	TP53
Oncogene Induced Senescence	1	112.0×	0.016	TP53

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
negative regulation of helicase activity	1	5617.3×	0.004	TP53
regulation of phospholipid catabolic process	1	5617.3×	0.004	IDH1
cellular response to actinomycin D	1	5617.3×	0.004	TP53
regulation of intrinsic apoptotic signaling pathway by p53 class mediator	1	5617.3×	0.004	TP53
negative regulation of G1 to G0 transition	1	5617.3×	0.004	TP53
glyoxylate cycle	1	2808.7×	0.004	IDH1
positive regulation of mitochondrial membrane permeability	1	2808.7×	0.004	TP53
regulation of phospholipid biosynthetic process	1	2808.7×	0.004	IDH1
oligodendrocyte apoptotic process	1	2808.7×	0.004	TP53
negative regulation of glucose catabolic process to lactate via pyruvate	1	2808.7×	0.004	TP53
negative regulation of pentose-phosphate shunt	1	2808.7×	0.004	TP53
obsolete homolactic fermentation	1	1872.4×	0.004	TP53
signal transduction by p53 class mediator	1	1872.4×	0.004	TP53
negative regulation of miRNA processing	1	1872.4×	0.004	TP53
intrinsic apoptotic signaling pathway in response to hypoxia	1	1872.4×	0.004	TP53
regulation of fibroblast apoptotic process	1	1872.4×	0.004	TP53
T cell proliferation involved in immune response	1	1404.3×	0.004	TP53
positive regulation of programmed necrotic cell death	1	1404.3×	0.004	TP53
oxidative stress-induced premature senescence	1	1404.3×	0.004	TP53
B cell lineage commitment	1	1123.5×	0.004	TP53
T cell lineage commitment	1	1123.5×	0.004	TP53
isocitrate metabolic process	1	1123.5×	0.004	IDH1
NADPH regeneration	1	1123.5×	0.004	IDH1
mRNA transcription	1	1123.5×	0.004	TP53
positive regulation of RNA polymerase II transcription preinitiation complex assembly	1	1123.5×	0.004	TP53
positive regulation of thymocyte apoptotic process	1	1123.5×	0.004	TP53
cellular response to UV-C	1	1123.5×	0.004	TP53
regulation of mitochondrial membrane permeability involved in apoptotic process	1	936.2×	0.005	TP53
viral process	1	802.5×	0.005	TP53
mitochondrial DNA repair	1	802.5×	0.005	TP53

Therapeutics

Drug target analysis

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

Druggability breadth: 3 of 3 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

Symbol	Example approved molecule
TP53	NITROFURANTOIN
IDH1	ENASIDENIB

Top cohort targets by molecule count

Symbol	Molecules	Max phase
TP53	196	4
IDH1	10	4
MGMT	2	3

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
NITROFURANTOIN	4	TP53
DIOSMIN	4	TP53
VERTEPORFIN	4	TP53
CANDESARTAN CILEXETIL	4	TP53
DIENESTROL	4	TP53
CLOTRIMAZOLE	4	TP53
COLCHICINE	4	TP53
NABUMETONE	4	TP53
SALMETEROL XINAFOATE	4	TP53
AMIODARONE HYDROCHLORIDE	4	TP53
FURAZOLIDONE	4	TP53
AMOXAPINE	4	TP53
RALOXIFENE HYDROCHLORIDE	4	TP53
NICARDIPINE HYDROCHLORIDE	4	TP53
SULCONAZOLE NITRATE	4	TP53
PYRITHIONE ZINC	4	TP53
LACTIC ACID	4	TP53
OXYMETHOLONE	4	TP53
CHLOROXINE	4	TP53
PROPIOLACTONE	4	TP53
CLOMIPRAMINE HYDROCHLORIDE	4	TP53
PHENYL AMINOSALICYLATE	4	TP53
THIORIDAZINE HYDROCHLORIDE	4	TP53
AMITRIPTYLINE HYDROCHLORIDE	4	TP53
ETHOPROPAZINE HYDROCHLORIDE	4	TP53
MECHLORETHAMINE HYDROCHLORIDE	4	TP53
ECONAZOLE NITRATE	4	TP53
TRIFLUPROMAZINE HYDROCHLORIDE	4	TP53
PROCHLORPERAZINE EDISYLATE	4	TP53
DEQUALINIUM CHLORIDE	4	TP53

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 2.

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

Symbol	Assays	Type breakdown
TP53	869	Binding:775, ADMET:83, Functional:10, Toxicity:1
IDH1	488	Binding:475, Functional:12, ADMET:1
MGMT	86	Binding:84, ADMET:2

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
IDH1	1.1.1.42	isocitrate dehydrogenase (NADP+)
MGMT	2.1.1.63	methylated-DNA-[protein]-cysteine S-methyltransferase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

Symbol	ChEMBL assays
TP53	869
IDH1	488

Pharmacogenomics

Cohort genes with a PharmGKB record: 3; with CPIC/DPWG dosing guidelines: 0.

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

Chemical tractability of cohort targets

30 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.

Compound	Max phase	Cohort target (bioactivity)
NITROFURANTOIN	4	TP53
DIOSMIN	4	TP53
VERTEPORFIN	4	TP53
CANDESARTAN CILEXETIL	4	TP53
DIENESTROL	4	TP53
CLOTRIMAZOLE	4	TP53
COLCHICINE	4	TP53
NABUMETONE	4	TP53
SALMETEROL XINAFOATE	4	TP53
AMIODARONE HYDROCHLORIDE	4	TP53
FURAZOLIDONE	4	TP53
AMOXAPINE	4	TP53
RALOXIFENE HYDROCHLORIDE	4	TP53
NICARDIPINE HYDROCHLORIDE	4	TP53
SULCONAZOLE NITRATE	4	TP53
PYRITHIONE ZINC	4	TP53
LACTIC ACID	4	TP53
OXYMETHOLONE	4	TP53
CHLOROXINE	4	TP53
PROPIOLACTONE	4	TP53
CLOMIPRAMINE HYDROCHLORIDE	4	TP53
PHENYL AMINOSALICYLATE	4	TP53
THIORIDAZINE HYDROCHLORIDE	4	TP53
AMITRIPTYLINE HYDROCHLORIDE	4	TP53
ETHOPROPAZINE HYDROCHLORIDE	4	TP53
MECHLORETHAMINE HYDROCHLORIDE	4	TP53
ECONAZOLE NITRATE	4	TP53
TRIFLUPROMAZINE HYDROCHLORIDE	4	TP53
PROCHLORPERAZINE EDISYLATE	4	TP53
DEQUALINIUM CHLORIDE	4	TP53

Druggability pyramid

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

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	2	TP53, IDH1
B	Phased (≥1) drug, not yet approved	1	MGMT
C	Druggable family + PDB, no drug	0
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	0

Undrugged target profiles

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

Clinical trials & evidence

Clinical trials

Clinical trials: 86.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	27
PHASE2	25
PHASE1	20
PHASE3	6
PHASE1/PHASE2	4
EARLY_PHASE1	3
PHASE2/PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01649830	PHASE3	RECRUITING	Efficacy of Post-radiation Adjuvant Temozolomide Chemotherapy in Residue Low-grade Glioma
NCT04702581	PHASE3	RECRUITING	A Randomized Trial of Delayed Radiotherapy in Patients Low-grade Oligodendrogliomas Requiring a Treatment Other Than Surgery
NCT05303519	PHASE3	RECRUITING	SIGMA (Safusidenib in IDH1 Mutant Glioma Maintenance)
NCT05331521	PHASE3	RECRUITING	A Clinical Study to Improve Brain Function and Quality of Life of Patients With Newly Diagnosed Brain Tumors (Gliomas).
NCT00717210	PHASE3	COMPLETED	Randomized Phase III Study of Sequential Radiochemotherapy of Anaplastic Glioma With PCV or Temozolomide
NCT00897377	PHASE3	TERMINATED	Treatment Strategy for Low-grade Gliomas
NCT04105374	PHASE2/PHASE3	WITHDRAWN	Testing the Addition of an Anti-cancer Viral Gene Therapy, Toca 511/Toca FC, to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed Glioblastoma
NCT03180502	PHASE2	ACTIVE_NOT_RECRUITING	Proton Beam or Intensity-Modulated Radiation Therapy in Preserving Brain Function in Patients With IDH Mutant Grade II or III Glioma
NCT04623931	PHASE2	RECRUITING	Chemotherapy and Radiation Therapy for the Treatment of IDH Wildtype Gliomas or Non-histological (Molecular) Glioblastomas
NCT05345002	PHASE2	RECRUITING	All-Trans Retinoic Acid (ATRA) Plus PD-1 Inhibition in Recurrent IDH-Mutant Glioma
NCT05512351	PHASE2	RECRUITING	Sintilimab (One Anti-PD-1 Antibody) Plus Low-dose Bevacizumab for ctDNAlevel- Relapse and Clinical-relapse Oligodendroglioma
NCT05956821	PHASE1/PHASE2	RECRUITING	Treatment of Relapsed/Refractory Intracranial Glioma in Patients Under 22 Years of Age
NCT06161974	PHASE2	RECRUITING	Study of Olutasidenib and Temozolomide in HGG
NCT07439172	PHASE2	NOT_YET_RECRUITING	Pre-Radiation Chemotherapy for Newly Diagnosed High-Grade Glioma.
NCT00004078	PHASE2	COMPLETED	Irinotecan in Treating Children With Refractory Solid Tumors
NCT00070525	PHASE2	COMPLETED	Tipifarnib in Treating Young Patients With Recurrent or Progressive High-Grade Glioma, Medulloblastoma, Primitive Neuroectodermal Tumor, or Brain Stem Glioma
NCT00095940	PHASE1/PHASE2	COMPLETED	Lapatinib in Treating Young Patients With Recurrent or Refractory Central Nervous System Tumors
NCT00165360	PHASE2	COMPLETED	Prolonged Daily Temozolomide for Low-Grade Glioma
NCT00360828	PHASE2	TERMINATED	Phase II Study of Irinotecan HCI for Recurrent Anaplastic Astrocytomas, Mixed Malignant Gliomas, and Oligodendrogliomas
NCT00381797	PHASE2	COMPLETED	Bevacizumab and Irinotecan in Treating Young Patients With Recurrent, Progressive, or Refractory Glioma, Medulloblastoma, Ependymoma, or Low Grade Glioma
NCT00389090	PHASE2	TERMINATED	A Phase II Study of Temozolomide and O6-Benzylguanine (O6-BG) in Patients With Temozolomide-Resistant Anaplastic Glioma
NCT00392171	PHASE2	COMPLETED	The Effects of Continuous 28-day (28/28) Temozolomide Chemotherapy in Subjects With Recurrent Malignant Glioma Who Have Failed the Conventional 5-day (5/28) Treatment (P04601)
NCT00575887	PHASE2	COMPLETED	Efficacy of Protracted Temozolomide in Patients With Progressive High Grade Glioma
NCT00683761	PHASE1/PHASE2	UNKNOWN	A Study of 131I-TM601 in Adults With Recurrent Malignant Glioma
NCT01462695	PHASE2	COMPLETED	Sunitinib Malate in Treating Younger Patients With Recurrent, Refractory, or Progressive Malignant Glioma or Ependymoma
NCT01609790	PHASE2	COMPLETED	Bevacizumab With or Without Trebananib in Treating Patients With Recurrent Brain Tumors
NCT01836549	PHASE2	TERMINATED	Imetelstat Sodium in Treating Younger Patients With Recurrent or Refractory Brain Tumors
NCT02023905	PHASE2	TERMINATED	Everolimus With and Without Temozolomide in Adult Low Grade Glioma
NCT02209428	PHASE2	UNKNOWN	A Prospective Cohort to Study the Effect of Temozolomide on IDH Mutational Low Grade Gliomas
NCT02372409	PHASE2	TERMINATED	Using MRI-Guided Laser Heat Ablation to Induce Disruption of the Peritumoral Blood Brain Barrier to Enhance Delivery and Efficacy of Treatment of Pediatric Brain Tumors
NCT02530320	PHASE2	COMPLETED	Safety and Efficacy of PD0332991 (Palbociclib), a Cyclin-dependent Kinase 4 and 6 Inhibitor, in Patients With Oligodendroglioma or Recurrent Oligoastrocytoma Anaplastic With the Activity of the Protein RB Preserved
NCT03014804	PHASE2	WITHDRAWN	Autologous Dendritic Cells Pulsed With Tumor Lysate Antigen Vaccine and Nivolumab in Treating Patients With Recurrent Glioblastoma
NCT03027388	PHASE2	COMPLETED	Protein Phosphatase 2A Inhibitor, in Recurrent Glioblastoma
NCT03649464	PHASE1/PHASE2	WITHDRAWN	Investigation of Oral OKN-007 in Recurrent High-grade Glioma Participants
NCT05297864	PHASE2	TERMINATED	PARP Inhibition for Gliomas (PI-4G or π4g)
NCT06439420	PHASE2	COMPLETED	CBT-I in Primary Brain Tumor Patients: Phase IIc Randomized Feasibility Pilot Trial
NCT03152318	PHASE1	ACTIVE_NOT_RECRUITING	A Study of the Treatment of Recurrent Malignant Glioma With rQNestin34.5v.2
NCT04541082	PHASE1	RECRUITING	Phase I Study of Oral ONC206 in Recurrent and Rare Primary Central Nervous System Neoplasms
NCT05484622	PHASE1	ACTIVE_NOT_RECRUITING	Study of Vorasidenib and Pembrolizumab Combination in Recurrent or Progressive IDH-1 Mutant Glioma
NCT05561374	PHASE1	ACTIVE_NOT_RECRUITING	Study of Safety and Pharmacokinetic Properties of Oral OKN-007 in Patients with Recurrent High-Grade Glioma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
TEMOZOLOMIDE	4	4
LAPATINIB	4	3
EDOTREOTIDE GALLIUM GA-68	4	1
FLUDEOXYGLUCOSE F 18	4	1
FLUORODOPA F 18	4	1
IMETELSTAT SODIUM	4	1
IRINOTECAN	4	1
KETOCONAZOLE	4	1
NIRAPARIB	4	1
OLUTASIDENIB	4	1
PALBOCICLIB	4	1
RETIFANLIMAB	4	1
SUNITINIB MALATE	4	1
VORASIDENIB	4	1
6-O-BENZYLGUANINE	3	2
VELIPARIB	3	2
CEDIRANIB MALEATE	3	1
CILENGITIDE	3	1
DISUFENTON SODIUM	3	1
TIPIFARNIB	3	1
TREBANANIB	3	1
HILTONOL	2	2
CHLOROTOXIN	2	1
LB-100	2	1
SAFUSIDENIB	2	1
VARLILUMAB	2	1
VOCIMAGENE AMIRETROREPVEC	2	1
ONC-206	1	1
PF-06840003	1	1
CHEMBL4228794	0	16

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 2 predictive associations from 2 curated evidence items; also 2 prognostic, 2 diagnostic.

Molecular subtype	Therapy	Effect	Level	CIViC
MGMT Underexpression	Temozolomide	Sensitivity/Response	CIViC B	EID2902
IDH1 R132H	AGI-5198	Sensitivity/Response	CIViC D	EID979