Olfactory neuroblastoma, also called esthesioneuroblastoma, is a very malignant, or cancerous, tumor believed to originate in the olfactory cells. The olfactory cells, located in the upper rear of the inside of the nose, are responsible for the sense of smell. This condition often responds well to radiation therapy, but the tumor has a high tendency to recur after excision.
Olfactory neuroblastoma is a relatively rare cancer. The cancer was first characterized in 1924, and there have been less than 1,000 recorded cases since then. It can cause loss of smell, taste, and vision, as well as facial disfigurement in advanced cases.
Despite its name, olfactory neuroblastoma is considered distinct from other neuroblastomas, because it does not originate in the sympathetic nervous system (SNS). The condition is controversial, because it is so rare that it cannot be easily studied and characterized. Some cases of esthesioneuroblastoma are extremely aggressive, resulting in death within a few months, while others grow slowly. Some patients have survived for 20 years with an olfactory neuroblastoma. No trends have been discovered regarding the tendency of esthesioneuroblastoma to affect individuals of a certain race, sex, or age, and the tumor does not appear to run in families.
As with other nasal cancers, the symptoms of olfactory neuroblastoma fall into six categories: nasal, facial, oral, ophthalmic, neurological, and cervical. Nasal symptoms include nasal obstruction, discharge, and polyps. Facial symptoms include swelling, pain, and lack of sensation. Oral symptoms include loose teeth, and the loss of teeth, as well as ulcers in the mouth. Ophthalmic symptoms can present as loss of vision, while cervical problems present as a mass in the neck. Nasal symptoms are the most common, and usually the first to appear.
Olfactory neuroblastoma is diagnosed through the observation of symptoms, followed by biopsy or imaging, such as a computed tomography (CT) scan, or magnetic resonance imaging (MRI). It is usually treated with surgery, followed by radiation. Chemotherapy is not common in the treatment of esthesioneuroblastoma.
The surgical treatment of esthesioneuroblastoma, like all surgeries, has risks. These include infection, meningitis, pneumocephalus, and blindness. There is a 10-15% change of developing one of these complications, and patients undergoing surgery for esthesioneuroblastoma have a five-year survival rate of 50-80%. The patient should be able to leave the hospital the day after surgery, and does not require extensive aftercare, but a lengthy follow-up is required due to the tumor's tendency to recur, sometimes years after surgery.
