Olfactory neuroblastoma is a rare and malignant tumor that originates in the olfactory cells responsible for smell. It can cause loss of smell, taste, and vision, and facial disfigurement. It is diagnosed through symptoms and imaging and is treated with surgery and radiation. Recurrence is common, and long-term follow-up is required.
Olfactory neuroblastoma, also called esthesioneuroblastoma, is a very malignant, or cancerous, tumor believed to originate in the olfactory cells. The olfactory cells, located at the upper back of the inside of the nose, are responsible for 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 tumor. The cancer was first characterized in 1924 and fewer than 1,000 cases have been recorded 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 months, while others grow slowly. Some patients have survived 20 years with olfactory neuroblastoma. No trends have been discovered regarding the tendency for 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, 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 tooth loss, as well as mouth ulcers. Ophthalmic symptoms can present as vision loss, 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 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 uncommon in the treatment of esthesioneuroblastoma.
Surgical treatment of esthesioneuroblastoma, like all surgeries, has risks. These include infections, meningitis, pneumocephalus and blindness. There is a 10-15% change in the development of any of these complications, and patients undergoing surgery for esthesioneuroblastoma have a 50-80% five-year survival rate. The patient should be able to leave the hospital the day after surgery and does not require extensive post-operative care, but a long follow-up is required due to the tendency of the tumor to recur, sometimes years later the intervention.
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