Olfactory neuroblastoma

Alternate names

Esthesioneuroblastoma

Definition

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.

Summary

The nasal cavity has nerves and other tissue that are responsible for the sense of smell. This kind of tumor begins in the nasal cavity and can grow into the nearby eyes and brain. Olfactory neuroblastoma can also spread to other parts of the body, such as the neck, lungs, and bones.

Epidemiology

Olfactory neuroblastoma is a rare type of cancer, making up only 3% of nasal cavity tumor cases. Olfactory neuroblastoma affects only about 1 person per 2.5 million each year. Olfactory neuroblastoma can form at any age but is most commonly seen in people ages 50 to 70 years old.

Cause

The cause of olfactory neuroblastoma is not well understood. No specific environmental or genetic causes have been confirmed, although comprehensive genetic testing of affected individuals has identified regions within chromosomes 2, 5, 6, 7, and 20 that may be involved.

Inheritance

Olfactory neuroblastoma is not thought to run in families.

Signs and symptoms

The most common symptom of olfactory neuroblastoma is blockage of the nasal passageway secondary to the tumor. Other signs and symptoms may include:

• Loss of smell (anosmia)
• Chronic sinus infections (sinusitis)
• Nasal bleeding
• Nasal discharge
• Pain
• Some symptoms might be associated with a spread (metastasis) of the tumor to other structures in the face.

These symptoms might include:

• Sinus pain and headache
• Visual changes
• Ear pain

Diagnosis

A diagnosis of olfactory neuroblastoma may be suspected based on presenting signs and symptoms and imaging studies such as MRI and CT scans. The diagnosis is confirmed through biopsy of the tumor.

• Imaging: If you have symptoms of olfactory neuroblastoma, your doctor will use imaging scans such as X-ray, CT, and MRI to look at where the tumor is and how big it is. They will also check for signs that the tumor has spread to other parts of the body.
• Biopsy: To check if the tumor is olfactory neuroblastoma your doctor will do a biopsy, taking a small sample from the tumor. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is.

Treatment

Treatment for each patient will be unique. You should go to an expert in olfactory neuroblastoma treatment to decide the best approach for your tumor. Treatment options to discuss with your doctor include:

• Surgery: Surgery is used to remove as much of the tumor as possible and some surrounding healthy tissue to improve the chances of removing all of the tumor cells.
• Radiation therapy: Radiation therapy is a treatment option for some olfactory neuroblastoma tumors.
• Radiation therapy may be the main treatment, or it may be used after surgery to help reduce the chances that the tumor will grow back.
• Chemotherapy: Chemotherapy may be used in combination with radiation after surgery to treat some olfactory neuroblastoma tumors or in cases where the tumor has spread to other parts of the body.

NIH genetic and rare disease info

• NIH info on Olfactory neuroblastoma

Olfactory neuroblastoma is a rare disease.

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