Chordoid glioma of the third ventricle

Summary

Chordoid glioma of the third ventricle (MONDO:0016706) is a cancer and 2 clinical trials. Top therapeutic interventions include dabrafenib and trametinib. A subtype of cerebral ventricle cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
• Clinical trials: 2

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Cases/families		80	Worldwide	Validated
Point prevalence	<1 / 1 000 000		Worldwide	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	chordoid glioma of the third ventricle
Mondo ID	MONDO:0016706
Orphanet	251674
DOID	DOID:3773, DOID:3774
NCIT	C5592
SNOMED CT	715900001
UMLS	C1322252
MedGen	232956
GARD	0020715
Anatomy (UBERON)	UBERON:0002286
Is cancer (heuristic)	yes

Also known as: chordoid glioma · chordoid glioma (morphologic abnormality) · chordoid glioma of 3rd ventricle · chordoid glioma of the 3rd ventricle · chordoid glioma of the third ventricle · chordoid glioma of the third ventricle (WHO grade II) · chordoid glioma of third ventricle · third ventricle chordoid glioma

Disease family

This is a subtype of cerebral ventricle cancer. 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: human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › nervous system cancer › central nervous system cancer › brain cancer › cerebral ventricle cancer › chordoid glioma of the third ventricle

Related subtypes (3): choroid plexus cancer, intraventricular meningioma, central neurocytoma

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: 2.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE4	1
PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03975829	PHASE4	RECRUITING	Pediatric Long-Term Follow-up and Rollover Study
NCT02684058	PHASE2	COMPLETED	Study of Efficacy and Safety of Dabrafenib in Combination With Trametinib in Pediatric Patients With BRAF V600 Mutation Positive LGG or Relapsed or Refractory HGG Tumors

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DABRAFENIB	4	2
TRAMETINIB	4	2
CHEMBL5433950	0	2