Olfactory neuroblastoma, or esthesioneuroblastoma, is a tumor that grows in the nasal cavity. 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.

What is neuroblastoma?

Neuroblastoma is a type of cancer that forms in nerve cells called neuroblasts. Neuroblasts are immature nerve tissue. They normally turn into working nerve cells. But in neuroblastoma, they form a tumor.

Neuroblastoma usually begins in the adrenal glands. You have two adrenal glands, one on top of each kidney. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the neck, chest or spinal cord.

What causes neuroblastoma?

Neuroblastoma is caused by mutations (changes) in genes. In most cases, the cause of the mutation is unknown. In some other cases, the mutation is passed from the parent to the child.

What are the symptoms of neuroblastoma?

Neuroblastoma often begins in early childhood. Sometimes it begins before a child is born.The most common symptoms are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone.They include

• A lump in the abdomen, neck or chest
• Bulging eyes
• Dark circles around the eyes
• Bone pain
• Swollen stomach and trouble breathing in babies
• Painless, bluish lumps under the skin in babies
• Inability to move a body part (paralysis)

How is neuroblastoma diagnosed?

To diagnose neuroblastoma, your child's health care provider will do various tests and procedures, which may include

• A medical history
• A neurological exam
• Imaging tests, such as x-rays, a CT scan an ultrasound, an MRI, or an MIBG scan. In an MIBG scan, a small amount of a radioactive substance is injected into a vein. It travels through the bloodstream and attaches itself to any neuroblastoma cells. A scanner detects the cells.
• Blood and urine tests
• Biopsy, where a sample of tissue is removed and examined under a microscope
• Bone marrow aspiration and biopsy, where bone marrow, blood, and a small piece of bone are removed for testing

What are the treatments for neuroblastoma?

The treatments for neuroblastoma include:

• Observation, also called watchful waiting, where the health care provider does not give any treatments until your child's signs or symptoms appear or change
• Surgery
• Radiation therapy
• Chemotherapy
• High-dose chemotherapy and radiation therapy with stem cell rescue. Your child will get high doses of chemotherapy and radiation. This kills the cancer cells, but it also kills healthy cells. So your child will get a stem cell transplant, usually of his or her own cells collected earlier. This helps to replace the healthy cells that were lost.
• Iodine 131-MIBG therapy, a treatment with radioactive iodine. The radioactive iodine collects in neuroblastoma cells and kills them with the radiation that is given off.
• Targeted therapy, which uses drugs or other substances that attack specific cancer cells with less harm to normal cells

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.

Scientists are always working to understand how cancer forms, but it can be hard to prove. So far, we do not yet know what causes olfactory neuroblastoma to form, but scientists have found that changes in some parts of the DNA, called chromosome 2, chromosome 5, chromosome 6, chromosome 7, and chromosome 20, may be involved.

No, olfactory neuroblastoma is not thought to run in families.

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

Olfactory neuroblastoma can cause several symptoms as the tumor grows, including:

• Nose bleeds
• Loss of the sense of smell
• Difficulty breathing through the nose
• Pain

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.

• Stage A-Tumors limited to the nasal cavity
• Stage B-Tumors that involve the nasal cavity and extend into the paranasal sinuses
• Stage C-Extension beyond the nasal cavity and the paranasal sinuses
• Stage D-Regional lymph node involvement or distant metastasis

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. You can contact MyPART for help finding experts near you.

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.

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:

• Where the tumor is in your body
• If the cancer has spread to other parts of your body
• How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis.

Doctors estimate olfactory neuroblastoma survival rates by how groups of people with olfactory neuroblastoma have done in the past. The survival rate varies depending on the stage of disease but is thought to range from 50-90%.