Ependymal tumor

disease

On this page

Also known as ependymal neoplasmependymal tumorsependymal tumoursependymomal tumorependymomal tumour

Summary

Ependymal tumor (MONDO:0003266) is a cancer (an umbrella term covering 6 Mondo subtypes) and 2 clinical trials. Top therapeutic interventions include ulixertinib. A subtype of glioma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

Classification: Cancer
Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
Umbrella term: 6 Mondo subtypes
Clinical trials: 2

Clinical features

Epidemiology

Prevalence records

5 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.2	Europe	Validated
Lifetime Prevalence	1-9 / 100 000	3.85	Europe	Validated
Point prevalence	1-9 / 100 000		Europe	Validated
Annual incidence	1-9 / 1 000 000	0.2	United Kingdom	Validated
Annual incidence	1-9 / 1 000 000	0.43	United States	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	ependymal tumor
Mondo ID	MONDO:0003266
EFO	EFO:1000027
Orphanet	301
NCIT	C6770
UMLS	C1333407
MedGen	232459
GARD	0016527
Is cancer (heuristic)	yes

Also known as: ependymal neoplasm · ependymal tumor · ependymal tumors · ependymal tumours · ependymomal tumor · ependymomal tumour

Disease family

This is a subtype of 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 etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › nervous system neoplasm › neuroepithelial neoplasm › glioma › ependymal tumor

Subtypes (6): ependymal tumor of brain, ependymoma, myxopapillary ependymoma, anaplastic ependymoma, ependymal tumor of spinal cord, RELA fusion-positive ependymoma

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
PHASE2	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03698994	PHASE2	ACTIVE_NOT_RECRUITING	Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial)
NCT04099797	PHASE1	RECRUITING	C7R-GD2.CAR T Cells for Patients With GD2-expressing Brain Tumors (GAIL-B)

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ULIXERTINIB	2	1

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.