Desmoplastic infantile ganglioglioma

Summary

Desmoplastic infantile ganglioglioma (MONDO:0022965) is a disease. Molecularly, EEF1G::ROS1 Fusion confers sensitivity to Entrectinib in Desmoplastic Infantile Ganglioglioma / Desmoplastic Infantile Astrocytoma (CIViC Level C). A subtype of mixed neuronal-glial tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Precision-medicine evidence (CIViC): 1 subtype–drug association

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	desmoplastic infantile ganglioglioma
Mondo ID	MONDO:0022965
DOID	DOID:0081259
NCIT	C4738
UMLS	C1321878
MedGen	231168
GARD	0008648
Is cancer (heuristic)	no

Also known as: Desmoplastic infantile ganglioglioma · DIG

Disease family

This is a subtype of mixed neuronal-glial tumor. 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 › mixed neuronal-glial tumor › desmoplastic infantile ganglioglioma

Related subtypes (11): ganglioneuroma, dysembryoplastic neuroepithelial tumor, extraventricular neurocytoma, gangliocytoma, desmoplastic infantile astrocytoma/ganglioglioma, ganglioglioma, papillary glioneuronal tumor, rosette-forming glioneuronal tumor of fourth ventricule, Lhermitte-Duclos disease, central neurocytoma, desmoplastic infantile 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: 0.

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 1 predictive associations from 1 curated evidence items.

Molecular subtype	Therapy	Effect	Level	CIViC
EEF1G::ROS1 Fusion	Entrectinib	Sensitivity/Response	CIViC C	EID11850