Childhood optic nerve glioma

Summary

Childhood optic nerve glioma (MONDO:0003932) is a cancer and 17 clinical trials. Top therapeutic interventions include selumetinib, carboplatin, and edotreotide gallium ga-68. A subtype of optic nerve glioma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 17

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	childhood optic nerve glioma
Mondo ID	MONDO:0003932
DOID	DOID:6576
NCIT	C7535
UMLS	C0278653
MedGen	124487
GARD	0009309
Is cancer (heuristic)	yes

Also known as: childhood optic tract glioma · childhood visual pathway glioma · glioma of childhood visual pathway · glioma of paediatric visual pathway · glioma of pediatric visual pathway · glioma of the childhood visual pathway · glioma of the paediatric visual pathway · glioma of the pediatric visual pathway · optic nerve glioma of childhood · paediatric optic nerve glioma · paediatric optic tract glioma · paediatric visual pathway glioma · pediatric optic nerve glioma · pediatric optic tract glioma · pediatric visual pathway glioma · visual pathway and hypothalamic glioma, childhood · visual pathway glioma

Disease family

This is a subtype of optic nerve glioma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › optic nerve disorder › optic nerve neoplasm › optic nerve glioma › childhood optic nerve glioma

Related subtypes (1): optic nerve astrocytoma

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

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 17.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	9
PHASE2	3
Not specified	3
PHASE3	1
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03871257	PHASE3	ACTIVE_NOT_RECRUITING	A Study of the Drugs Selumetinib Versus Carboplatin/Vincristine in Patients With Neurofibromatosis and Low-Grade Glioma
NCT01089101	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Selumetinib in Treating Young Patients With Recurrent or Refractory Low Grade Glioma
NCT00003477	PHASE2	COMPLETED	Antineoplaston Therapy in Treating Children With Visual Pathway Glioma
NCT00004078	PHASE2	COMPLETED	Irinotecan in Treating Children With Refractory Solid Tumors
NCT01553149	PHASE2	COMPLETED	Low-Dose or High-Dose Lenalidomide in Treating Younger Patients With Recurrent, Refractory, or Progressive Pilocytic Astrocytoma or Optic Pathway Glioma
NCT00012181	PHASE1	COMPLETED	Flavopiridol in Treating Children With Relapsed or Refractory Solid Tumors or Lymphomas
NCT00101270	PHASE1	COMPLETED	Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas
NCT00326664	PHASE1	COMPLETED	AZD2171 in Treating Young Patients With Recurrent, Progressive, or Refractory Primary CNS Tumors
NCT00638898	PHASE1	COMPLETED	Busulfan, Melphalan, Topotecan Hydrochloride, and a Stem Cell Transplant in Treating Patients With Newly Diagnosed or Relapsed Solid Tumor
NCT00929903	PHASE1	COMPLETED	Pazopanib Hydrochloride in Treating Young Patients With Solid Tumors That Have Relapsed or Not Responded to Treatment
NCT00994500	PHASE1	COMPLETED	Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma
NCT01088763	PHASE1	TERMINATED	Gamma-Secretase Inhibitor RO4929097 in Treating Young Patients With Relapsed or Refractory Solid Tumors, CNS Tumors, Lymphoma, or T-Cell Leukemia
NCT02415153	PHASE1	COMPLETED	Pomalidomide in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors
NCT02780804	PHASE1	COMPLETED	Entinostat in Treating Pediatric Patients With Recurrent or Refractory Solid Tumors
NCT00919750	Not specified	COMPLETED	Collecting and Storing Blood and Brain Tumor Tissue Samples From Children With Brain Tumors
NCT02175745	Not specified	TERMINATED	18F-FDOPA PET/CT or PET/MRI in Measuring Tumors in Patients With Newly-Diagnosed or Recurrent Gliomas
NCT02194452	Not specified	WITHDRAWN	Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
SELUMETINIB	4	3
CARBOPLATIN	4	1
EDOTREOTIDE GALLIUM GA-68	4	1
FLUORODOPA F 18	4	1
PAZOPANIB HYDROCHLORIDE	4	1
ALVOCIDIB	3	3
CEDIRANIB MALEATE	3	1
ENTINOSTAT	3	1
CHEMBL541887	0	1
CHEMBL4463209	0	1