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Olfactory Neuroblastoma

Olfactory neuroblastoma is a rare cancer of the upper part of the nasal cavity called the cribiform plate, which is a bone deep in the skull between the eyes, and above the ethmoid sinuses

Prevalence

<1 / 1 000 000

< 331

US Estimated

< 514

Europe Estimated

Age of Onset

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ICD-10

C30.0

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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5 Facts you should know

FACT

1

A rare cancer of the upper part of the nasal cavity

FACT

2

It accounts for about 5% of all cancers of the nasal cavity and paranasal sinuses

FACT

3

Is of neurocrest origin, arising from olfactory sensory cells in the olfactory epithelium

FACT

4

The most common signs and symptoms are nasal obstruction (70%) and epistaxis (50%)

FACT

5

It is a slow developing but malignant tumor with high recurrence rates because of its anatomical position

Olfactory Neuroblastoma is also known as...

Olfactory Neuroblastoma

Esthesioneuroblastoma

What’s your Rare IQ?

When is the incidence of olfactory neuroblastoma most common?

Common signs & symptoms

Loss of smell (anosmia)

Chronic sinus infections (sinusitis)

Nasal bleeding

Nasal discharge

Pain

Sinus pain and headache

Visual changes

Ear pain

Current treatments

A surgical removal of the tumor

Radiation therapy

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Phase 2 Study of Bintrafusp Alfa in Recurrent/Metastatic Olfactory Neuroblastoma (BARON).Olfactory neuroblastoma (ONB) is a rare cancer of the nasal cavity. At diagnosis, it is usually locally advanced. It tends to spread to the neck. Sometimes it spreads to the lungs and bones. Researchers want to find a better way to treat it. Objective: To learn if giving immunotherapy drug bintrafusp alfa can help ONB shrink or disappear.Phase 2Not yet recruitingDrug: Bintrafusp alfa / M7824Click here for more information
A Clinical Trial of Endoscopic Surgery Followed by Chemotherapy and Proton Radiation for the Treatment of Tumors in the Sinus and Nasal PassagesThis study is being done to test a new treatment plan for large tumors in the sinus or nasal cavity that will include endoscopic surgery plus chemotherapy and proton-beam radiation therapy.Phase 2Active, not recruitingDrug: Cisplatin|Radiation: Adjuvant Proton Radiotherapy|Procedure: Endoscopic Resection|Drug: cisplatin and etoposideClick here for more information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore Information
Bintrafusp alfa / M7824Bintrafusp alfa is a bifunctional fusion protein designed to simultaneously block two immunosuppressive pathways, TGF-β and PD-L1, within the tumor microenvironment. This bifunctional approach is thought to control tumor growth by potentially restoring and enhancing anti-tumor responses.Phase 2National Cancer Institute (NCI)|National Institutes of Health Clinical Center (CC)Click here for more informationClick here for more information
18F-fludeoxyglucose (18F-FDG)/18F-FPPRGD2a radiopharmaceutical agent used in positron emission tomographic (PET) imaging.Phase 1|Phase 2Sanjiv Sam Gambhir|National Cancer Institute (NCI)|Stanford UniversityClick here for more informationClick here for more information
doxorubicin hydrochloride|gemcitabine hydrochlorideBoth gemcitabine diphosphate (dFdCTP) and gemcitabine triphosphate (dFdCTP) inhibit processes required for DNA synthesis. Incorporation of dFdCTP into DNA is most likely the major mechanism by which gemcitabine causes cell death. Doxorubicin has antimitotic and cytotoxic activity through a number of proposed mechanisms of action: Doxorubicin forms complexes with DNA by intercalation between base pairs, and it inhibits topoisomerase II activity by stabilizing the DNA-topoisomerase II complex, preventing the religation portion of the ligation- ...Phase 2Medical University of South CarolinaClick here for more informationClick here for more information
Cisplatin|Radiation: Adjuvant Proton Radiotherapy|Procedure: Endoscopic Resection|Drug: cisplatin and etoposideA new treatment paradigm for large tumors in the sinus or nasal cavity that will include endoscopic surgery plus chemotherapy and proton-beam radiation therapy.Phase 2Memorial Sloan Kettering Cancer CenterClick here for more informationClick here for more information
Doxepin hydrochlorideDoxepin is a tricyclic antidepressant that widely used in the therapy of depression. Doxepin can cause mild and transient serum enzyme elevations but is a rare cause of clinically apparent acute cholestatic liver injury.


A dibenzoxepin tricyclic compound. It displays a range of pharmacological actions including maintaining adrenergic innervation. Its mechanism of action is not fully understood, but it appears to block reuptake of monoaminergic neurotransmitters into presynaptic terminals. It also possesses anticholinergic activity and modulates antagonism of histamine H(1)- and H(2)-receptors.
Phase 3Alliance for Clinical Trials in Oncology|National Cancer Institute (NCI)Click here for more informationClick here for more information