Esthesioneuroblastoma

Summary

Esthesioneuroblastoma (MONDO:0016029) is a disease and 7 clinical trials. Top therapeutic interventions include ibuprofen, lutetium oxodotreotide lu-177, and tarlatamab. A subtype of central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Clinical trials: 7

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.02	Europe	Validated
Point prevalence	<1 / 1 000 000		Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	esthesioneuroblastoma
Mondo ID	MONDO:0016029
Orphanet	1957
SNOMED CT	422886007
UMLS	C0206717
MedGen	60217
GARD	0002197
Is cancer (heuristic)	no

Data availability: 5 cell lines.

Disease family

This is a subtype of central nervous system Ewing sarcoma/peripheral primitive neuroectodermal 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 › cancer › nervous system cancer › central nervous system cancer › central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor › esthesioneuroblastoma

Related subtypes (3): medulloepithelioma, ependymoblastoma, embryonal tumor with multilayered rosettes

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: 7.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	3
PHASE1/PHASE2	2
PHASE4	1
PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04081701	PHASE4	RECRUITING	68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors.
NCT06607692	PHASE1/PHASE2	RECRUITING	Study in Children and Adolescents of 177Lu-DOTATATE (Lutathera®) Combined With the PARP Inhibitor Olaparib for the Treatment of Recurrent or Relapsed Solid Tumours Expressing Somatostatin Receptor (SSTR) (LuPARPed).
NCT06814496	PHASE1/PHASE2	RECRUITING	Radiation Combined With BIspecific T-Cell Engager in DLL3 Expressing Tumors
NCT05012098	PHASE2	COMPLETED	Phase 2 Study of Bintrafusp Alfa in Recurrent/Metastatic Olfactory Neuroblastoma (BARON).
NCT04087902	Not specified	ACTIVE_NOT_RECRUITING	Long-Term Longitudinal QoL in Patients Undergoing EEA
NCT04755205	Not specified	RECRUITING	A Natural History Study of Children and Adults With Olfactory Neuroblastoma
NCT01586767	Not specified	UNKNOWN	Intensity-Modulated or Proton Radiation Therapy for Sinonasal Malignancy

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
IBUPROFEN	4	1
LUTETIUM OXODOTREOTIDE LU-177	4	1
TARLATAMAB	4	1
BINTRAFUSP ALFA	3	1