Disease · MONDO_0006329

olfactory neuroblastoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. olfactory neuroblastoma. Diseases, record MONDO_0006329. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0006329

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: olfactory neuroblastoma; Open Targets
Open Targets ID: MONDO_0006329; Open Targets
Other names: Asthesioneuroblastoma, Asthesioneuroblastoma (morphologic abnormality), Esthesioneuroblastoma (morphologic abnormality), Esthesioneuroblastoma, Olfactory, Esthesioneuroepithelioma (morphologic abnormality), Esthesioneuroepithelioma [dup] (morphologic abnormality), Olfactory Esthesioneuroblastoma||paranasal sinus Olfactory neuroblastoma, olfactory esthesioneuroblastoma, olfactory neuroepithelioma; Open Targets
Disease Ontology: DOID:369; Disease Ontology
NCI Thesaurus: C3789; NCI Thesaurus
Highest stage of its drugs: Phase 2, for 10 of its 10 drugs, from ClinicalTrials.gov; Open Targets

Drugs10

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
BINTRAFUSP ALFA	CHEMBL4298024	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CISPLATIN	CHEMBL11359	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ENASIDENIB	CHEMBL3989908	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
GEMCITABINE	CHEMBL888	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
LUTETIUM OXODOTREOTIDE LU-177	CHEMBL4297545	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
RIVOCERANIB	CHEMBL3186534	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ZANZALINTINIB	CHEMBL5314428	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets28

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
CD274	Programmed cell death 1 ligand 1	1	Programmed cell death 1 ligand 1 binding agent	Phase 2	https://conductscience.org/library/genes/29126	Drugs and targets
IDH2	Isocitrate dehydrogenase [NADP], mitochondrial	1	Isocitrate dehydrogenase [NADP], mitochondrial inhibitor	Phase 2	https://conductscience.org/library/genes/3418	Drugs and targets
KDR	Vascular endothelial growth factor receptor 2	1	Vascular endothelial growth factor receptor 2 inhibitor	Phase 2	https://conductscience.org/library/genes/3791	Drugs and targets
PDCD1	Programmed cell death protein 1	1	Programmed cell death protein 1 inhibitor	Phase 2	https://conductscience.org/library/genes/5133	Drugs and targets
POLA1	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/5422	Drugs and targets
POLA2	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/23649	Drugs and targets
POLD1	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/5424	Drugs and targets
POLD2	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/5425	Drugs and targets
POLD3	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/10714	Drugs and targets
POLD4	DNA polymerase (alpha/delta/epsilon)	1	DNA polymerase (alpha/delta/epsilon) inhibitor	Phase 2	https://conductscience.org/library/genes/57804	Drugs and targets

1 to 10 of 28

See all 28 targets

Trials7

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT05012098	Phase 2 Study of Bintrafusp Alfa in Recurrent/Metastatic Olfactory Neuroblastoma (BARON).	Phase 2	11	2025-08-08	Olfactory Neuroblastoma, Esthesioneuroblastoma, Neoplasm of the Nasal Cavity	BINTRAFUSP ALFA	Its record names the disease	ClinicalTrials.gov
NCT03454763	Optimizing the Interval Between Cycles of PRRT with 177lu-dotatate in Sstr2 Positive Tumors	Phase 2	618		Neuroendocrine Tumors	LUTETIUM OXODOTREOTIDE LU-177	Its record names the disease	ClinicalTrials.gov
NCT06176989	Enasidenib in IDH2-Mutated Malignant Sinonasal and Skull Base Tumors	Phase 2	40		Metastatic Chondrosarcoma, Locally Advanced Chondrosarcoma, Metastatic Sinonasal Adenocarcinoma, Locally Advanced Sinonasal Adenocarcinoma, Metastatic Large-cell Neuroendocrine Carcinoma, Locally Advanced Large-cell Neuroendocrine Carcinoma, Metastatic Olfactory Neuroblastoma, Locally Advanced Olfactory Neuroblastoma, Metastatic Sinonasal Undifferentiated Carcinoma, Locally Advanced Sinonasal Undifferentiated Carcinoma	ENASIDENIB	Its record names the disease	ClinicalTrials.gov
NCT06255210	Efficacy and Safety of Induction Chemotherapy for Olfactory Neuroblastoma (ESICON)	Phase 2	25		Olfactory Neuroblastoma	CISPLATIN, CYCLOPHOSPHAMIDE, ETOPOSIDE, GEMCITABINE	Its record names the disease	ClinicalTrials.gov
NCT07527169	A Phase 2 Study Of Zanzalintinib For Patients With Recurrent Or Metastatic Olfactory Neuroblastoma	Phase 2	16		Metastatic Olfactory Neuroblastoma	ZANZALINTINIB	Its record names the disease	ClinicalTrials.gov
NCT07100704	Recurrent/Metastatic Olfactory Neuroblastoma: Evaluating the Efficacy and Safety of Nivolumab	Phase 2	14		Recurrent/ Metastatic Olfactory Neuroblastoma	NIVOLUMAB	Its record names the disease	ClinicalTrials.gov
NCT06308575	A Phase II Study of Rivoceranib for Patients With Recurrent or Metastatic Olfactory Neuroblastoma	Phase 2	0		Metastatic Olfactory Neuroblastoma, Recurrent Olfactory Neuroblastoma	RIVOCERANIB	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials3

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
Cohort 1/Arm 1: Treatment With Bintrafusp Alfa - Immune Checkpoint vs Cohort 2/Arm 1: Treatment With Bintrafusp Alfa - Immune Checkpoint Blockade Resistant (ICB-R): Number of Participants With Serious and/or Non-serious Adverse Events Assessed by the Common Terminology Criteria for Adverse Events (CTCAE v5.0)	9 of 9	2 of 2	11	NCT05012098	Trial comparisons
Cohort 1/Arm 1: Treatment With Bintrafusp Alfa vs Cohort 2/Arm 1: Treatment With Bintrafusp Alfa: deaths from any cause	1 of 9	1 of 2	11	NCT05012098	Trial comparisons
Cohort 1/Arm 1: Treatment With Bintrafusp Alfa vs Cohort 2/Arm 1: Treatment With Bintrafusp Alfa: serious adverse events	3 of 9	2 of 2	11	NCT05012098	Trial comparisons

Papers from its trials5

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
42307670	DERIVED	1	PubMed
39571544	DERIVED	1	PubMed
39500789	DERIVED	1	PubMed
38581469	DERIVED	1	PubMed
34980502	DERIVED	1	PubMed

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0006329

GET https://conductscience.org/api/diseases/v1/MONDO_0006329/targets?page=2

API reference For AI assistants