Olfactory neuroblastoma

Summary

Olfactory neuroblastoma (MONDO:0006329) is a disease and 6 clinical trials. Top therapeutic interventions include enasidenib, ibuprofen, and bintrafusp alfa. A subtype of malignant cranial nerve neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 6

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	olfactory neuroblastoma
Mondo ID	MONDO:0006329
EFO	EFO:1000407
DOID	DOID:369
ICD-11	2007774165
NCIT	C3789
GARD	0024371
Is cancer (heuristic)	no

Also known as: Asthesioneuroblastoma · Asthesioneuroblastoma (morphologic abnormality) · olfactory esthesioneuroblastoma · olfactory neuroblastoma · olfactory neuroepithelioma

Data availability: 5 cell lines.

Disease family

An umbrella term covering 1 Mondo subtype.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › peripheral nervous system disorder › peripheral nervous system neoplasm › cranial nerve neoplasm › malignant cranial nerve neoplasm › olfactory neuroblastoma

Related subtypes (4): malignant neoplasm of acoustic nerve, oculomotor nerve cancer, optic nerve astrocytoma, malignant olfactory nerve neoplasm

Subtypes (1): nasal cavity olfactory neuroblastoma

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

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Cisplatin, Etoposide, Gemcitabine, Rivoceranib.

Clinical trials & evidence

Clinical trials

Clinical trials: 6.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	5
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06176989	PHASE2	RECRUITING	Enasidenib in IDH2-Mutated Malignant Sinonasal and Skull Base Tumors
NCT06255210	PHASE2	RECRUITING	Efficacy and Safety of Induction Chemotherapy for Olfactory Neuroblastoma (ESICON)
NCT07527169	PHASE2	NOT_YET_RECRUITING	A Phase 2 Study Of Zanzalintinib For Patients With Recurrent Or Metastatic Olfactory Neuroblastoma
NCT05012098	PHASE2	COMPLETED	Phase 2 Study of Bintrafusp Alfa in Recurrent/Metastatic Olfactory Neuroblastoma (BARON).
NCT06308575	PHASE2	WITHDRAWN	A Phase II Study of Rivoceranib for Patients With Recurrent or Metastatic Olfactory Neuroblastoma
NCT04755205	Not specified	RECRUITING	A Natural History Study of Children and Adults With Olfactory Neuroblastoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ENASIDENIB	4	1
IBUPROFEN	4	1
BINTRAFUSP ALFA	3	1
ZANZALINTINIB	3	1