Esthesioneuroblastoma
Esthesioneuroblastoma is a rare type of cancer that starts in the upper part of the inside of the nose, known as the nasal cavity. Esthesioneuroblastoma also is called olfactory neuroblastoma.
This cancer usually affects adults. But it can occur at any age. Esthesioneuroblastoma usually begins as a growth of cells, called a tumor, inside the nose. It might grow and go into the sinuses, eyes and brain. It also can spread to other parts of the body.
People with esthesioneuroblastoma can lose their sense of smell. They might get nosebleeds. And they might have trouble breathing through their nose as the tumor grows.
Esthesioneuroblastoma treatment usually includes surgery. Often, radiation and chemotherapy are part of the treatment as well.
Esthesioneuroblastoma symptoms include:
- Loss of the sense of smell.
- Frequent nosebleeds.
- Difficulty breathing through the nose.
As the cancer grows, it might cause eye pain, loss of vision, ear pain and headaches.
When to see a doctor
Make an appointment with your healthcare team if you have lasting symptoms that worry you.
Experts haven’t found the exact cause of esthesioneuroblastoma. In general, cancer happens when cells get changes in their DNA. A cell’s DNA holds the instructions that tell a cell what to do. The changes tell the cells to make many more cells quickly. The changes give the cells the ability to keep living when healthy cells would naturally die. This causes too many cells.
The cells might form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cells can break away and spread to other parts of the body.
Healthcare professionals haven’t found many risk factors for esthesioneuroblastoma. This cancer can happen at any age. But it’s more common in adults.
Complications of esthesioneuroblastoma may include:
- Cancer that grows into nearby organs and tissues. Esthesioneuroblastoma can grow and get into the sinuses, eyes and brain.
- Spread of the cancer, known as metastasis. Esthesioneuroblastoma can spread to other parts of the body, such as the lymph nodes, bone marrow, lungs, liver, skin and bones.
An esthesioneuroblastoma diagnosis might involve:
- Physical exam. A member of your healthcare team may take a history of your symptoms and look at your eyes, nose, and head and neck.
- Endoscopic exam. A healthcare professional may put a thin, flexible tube, known as an endoscope, into the nose. The tube has a camera attached that allows the healthcare professional to look at the cancer and see how large it is.
- Imaging tests. Imaging tests take pictures of the inside of the body. They can show the size of the cancer, exactly where it is and whether it has spread. Imaging tests might include magnetic resonance imaging (MRI) scans, computerized tomography (CT) scans and positron emission tomography (PET) scans.
- Removing a sample of tissue for testing, also called a biopsy. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A biopsy might be done by putting a special tool into the nose to remove a small piece of the cancer. This procedure can often be done in a healthcare professional’s office.
Diagnosing esthesioneuroblastoma can be hard. It’s rare, and it can look like other cancers that occur in the head, neck or nose. Testing can show if the cancer is esthesioneuroblastoma and it can give other information about the cancer that will help in making a treatment plan.
Esthesioneuroblastoma treatment usually involves surgery to remove the cancer. Other treatments include radiation and chemotherapy.
Treatment for esthesioneuroblastoma usually involves a team of experts with different specialties. The team might include:
- Surgeons who operate on the nervous system, known as neurosurgeons.
- Head and neck surgeons.
- Doctors who use radiation to treat cancer, known as radiation oncologists.
- Doctors who use medicine to treat cancer, known as medical oncologists.
If the person with esthesioneuroblastoma is a child, the team also might include specialists in pediatric surgery and oncology.
Surgery
The type of surgery depends on where the tumor is and how large it is. Surgery might involve:
- Removing the part of the tumor that’s in the nose. This is usually done using a thin, flexible tube, known as an endoscope. The tube has a camera that lets the surgeon see the cancer. Special surgical tools passed through the endoscope help with removing the cancer and nearby tissue.
- Opening the skull to get to the tumor, known as a craniotomy. This procedure involves removing a small piece of skull. That allows the surgeon to remove the tumor from the brain.
Surgery complications might include spinal fluid leaking into the nose, infection and vision problems.
Radiation therapy
Radiation therapy uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. People with esthesioneuroblastoma often have radiation therapy after surgery to kill any cancer cells that might remain in the head and neck.
If surgery isn’t possible, radiation therapy can be used alone or with chemotherapy.
Chemotherapy
Chemotherapy uses strong medicines to kill cancer cells. In people with esthesioneuroblastoma, chemotherapy might be used with radiation therapy after surgery to kill cancer cells that remain.
References
- Winn HR, ed. Esthesioneuroblastoma. In: Youmans and Winn Neurological Surgery. 8th ed. Elsevier; 2023. https://www.clinicalkey.com. Accessed Jan. 25, 2023.
- Snyderman C, et al. Olfactory neuroblastoma (esthesioneuroblastoma). https://www.uptodate.com/contents/search. Accessed Jan. 25, 2023.
- Karp EE, et al. Esthesioneuroblastoma (olfactory neuroblastoma): Overview and extent of surgical approach and skull base resection. Journal of Neurological Surgery Reports. 2022; doi:10.1055/s-0042-1753519.
- Childhood esthesioneuroblastoma treatment (PDQ) — Health professional version. National Cancer Institute. https://www.cancer.gov/types/head-and-neck/hp/child/esthesioneuroblastoma-treatment-pdq. Accessed Jan. 25, 2023.
- Ami TR. Allscripts EPSi. Mayo Clinic. Feb. 1, 2023.
- Childhood esthesioneuroblastoma treatment (PDQ) — Patient version. National Cancer Institute. https://www.cancer.gov/types/head-and-neck/patient/child/esthesioneuroblastoma-treatment-pdq. Accessed Jan. 26, 2023.
- Ernst E. Complementary and alternative therapies for cancer. https://www.uptodate.com/contents/search. Accessed Jan. 25, 2023.
- Fiani B, et al. Esthesioneuroblastoma: A comprehensive review of diagnosis, management, and current treatment options. World Neurology. 2019; doi: 10.1016/j.wneu.2019.03.014.
- Olfactory neuroblastoma. National Cancer Institute. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/other-rare-tumors/olfactory-neuroblastoma. Accessed Dec. 16, 2024.
- Lerner DK, et al. Personalized approach to olfactory neuroblastoma care. Journal of Personalized Medicine. 2024; doi:10.3390/jpm14040423.
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