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A rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases.
Biomarker and diagnostic research for esthesioneuroblastoma has been reported in the published literature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
11 clinical trials registered, 8 recruiting. Interventions under study include drug therapy, other interventions, and procedural interventions. Pipeline includes 1 PHASE4, 4 PHASE2, 3 PHASE1. Research is primarily sponsored by academic and government institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT06607692](https://clinicaltrials.gov/study/NCT06607692) |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 4:30 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
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). |
PHASE1 |
Fundación de investigación HM |
RECRUITING |
[NCT06255210](https://clinicaltrials.gov/study/NCT06255210) | Efficacy and Safety of Induction Chemotherapy for Olfactory Neuroblastoma (ESICON) | PHASE2 | Hongmeng Yu | RECRUITING |
[NCT07100704](https://clinicaltrials.gov/study/NCT07100704) | Recurrent/Metastatic Olfactory Neuroblastoma: Evaluating the Efficacy and Safety of Nivolumab | PHASE2 | National Cancer Center Hospital East | RECRUITING |
[NCT04755205](https://clinicaltrials.gov/study/NCT04755205) | A Natural History Study of Children and Adults With Olfactory Neuroblastoma | — | National Cancer Institute (NCI) | RECRUITING |
[NCT06176989](https://clinicaltrials.gov/study/NCT06176989) | Enasidenib in IDH2-Mutated Malignant Sinonasal and Skull Base Tumors | PHASE2 | National Cancer Institute (NCI) | RECRUITING |
141 publications have been identified in PubMed for esthesioneuroblastoma. Research spans Review / Meta-Analysis (23%), Case Report / Case Series (22%), and Clinical Trial Publication (16%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 30 | 23% |
Patient case studies | 28 | 22% |
Clinical study results | 21 | 16% |
Testing and diagnosis research | 13 | 10% |
Laboratory research | 13 | 10% |
Disease patterns and progression | 12 | 9% |
Other research | 6 | 5% |
New treatment approaches | 6 | 5% |
Chen SF (2026). [PMID: 41467362](https://pubmed.ncbi.nlm.nih.gov/41467362/). *Head Neck*. [Clinical Trial Publication]
Stefanovic M (2026). [PMID: 40856008](https://pubmed.ncbi.nlm.nih.gov/40856008/). *Laryngoscope*. [Diagnostic / Biomarker]
Takeda T (2026). [PMID: 42095030](https://pubmed.ncbi.nlm.nih.gov/42095030/). *J Neurol Surg B Skull Base*. [Diagnostic / Biomarker]
Sidhu AS (2026). [PMID: 40539965](https://pubmed.ncbi.nlm.nih.gov/40539965/). *Ophthalmic Plast Reconstr Surg*. [Review / Meta-Analysis]
Correa-Correa V (2026). [PMID: 42232427](https://pubmed.ncbi.nlm.nih.gov/42232427/). *Surg Neurol Int*. [Clinical Trial Publication]
Nishida A (2026). [PMID: 42203325](https://pubmed.ncbi.nlm.nih.gov/42203325/). *Anticancer Res*. [Clinical Trial Publication]
Castro Toscano LA (2026). [PMID: 41777935](https://pubmed.ncbi.nlm.nih.gov/41777935/). *Cureus*. [Clinical Trial Publication]
Olson E (2026). [PMID: 41503426](https://pubmed.ncbi.nlm.nih.gov/41503426/). *J Neurol Surg B Skull Base*. [Basic Science / Preclinical]
Jauhiainen MK (2026). [PMID: 41874350](https://pubmed.ncbi.nlm.nih.gov/41874350/). *Neuropathology*. [Basic Science / Preclinical]
Xue E (2026). [PMID: 41221671](https://pubmed.ncbi.nlm.nih.gov/41221671/). *Head Neck*. [Clinical Trial Publication]