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Esthesioneuroblastoma

condition

Definition

A esthesioneuroblastoma, also known as an olfactory neuroblastoma (or ONB), is a cancerous tumor of the small nerve cells along the roof the nose. These nerve cells provide the sense of smell. Often, these tumors include both the nose and the nerves and portions of brain just above the nose inside the head.

The disease most often affects patients between 40 and 70 years of age and affects slightly more men than women.

Unlike many other tumors that are staged according to tumor size on scale of 1-4, this condition is staged using the Kadish system:

  • Kadish A tumors are confined to the nasal cavity
  • Kadish B tumors extend into the nearby nasal sinuses
  • Kadish C tumors are more advanced and extend into the eye socket, skull base, brain, neck lymph nodes or other areas of the body

Symptoms

The most common symptoms include nasal obstruction (difficulty breathing through the nose) or nose bleeds. These symptoms often start gradually and are mistaken for other, more common causes.

This type of tumor is generally slow growing. As a result, 83% of patients with esthesioneuroblastoma already have tumor extending beyond the nose (into the brain or other areas of the body) at the time of diagnosis.

Signs that the tumor has spread may include loss of smell, vision loss, eye pain or tearing, ear pain and severe headaches.

diagnosis

Diagnosis

Diagnosis of an ethesioneuroblastoma usually requires a biopsy, which is often performed at the doctor’s office by an otolaryngologist (ear, nose and throat specialist). 

Once a diagnosis is made, your doctor will also order CT (computed tomography) and MRI (magnetic resonance imaging) tests to help evaluate the location, size and extent of the tumor.

treatment

Treatment

Esthesioneuroblastoma is treated with a combined approach that includes chemotherapy, radiation therapy and surgery. 

Surgery

Surgery is usually needed for a long-term cure. A team approach is often used, with head and neck surgeons working directly with neurosurgeons to remove all portions of the tumor.

At UVA, we use the craniofacial approach, which has become the gold standard for treatment. This treatment includes making incisions on the face (next to the nose) to remove the portion of the tumor from the nose, as well as incisions along the top of the head that allow neurosurgeons to remove portions of the tumor from the brain. This treatment is often used in combination with radiation and chemotherapy.

A less invasive approach can also be used where small cameras (endoscopes) and small instruments are inserted through the nostril to avoid facial incisions. This technique is often combined with an incision across the top of the head to fully remove any portion of the tumor that is in the brain.

Radiation Therapy

Radiation therapy alone is used in some cases, but it usually isn't as successful without surgery. At UVA, radiation is often used before surgery to shrink the tumor.

Chemotherapy

At UVA, we also use chemotherapy to treat esthesioneuroblastoma in patients with the most extensive disease.

prevention

Prevention

There are no known causes or risk factors for esthesioneuroblastoma.

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