Esthesioneuroblastoma

Esthesioneuroblastoma, also termed as olfactory neuroblastoma, is a rare malignant (non-cancerous) tumor that originates from the upper part your nasal cavity that is along the roof of your nose. It affects the nerve cells that provide the sense of smell. Sometimes, these tumors involve the nose, nerves, and also the part of the brain present just above your nose.

According to a study, the incidence of esthesioneuroblastoma is 0.4 percent per 1,000,000 people. It is estimated that nearly 1200 cases of esthesioneuroblastoma were reported since 1924. But, 80 percent of those were reported in the past 25 years. This type of cancer may occur in individuals of any age. Mostly, it affects the people aged between 40 and 70 years. Women are prone to esthesioneuroblastoma than men.

Symptoms

TThe growth of the olfactory tumor is very slow, although, some types of tumors grow aggressively. The average time required for the onset of symptoms and the initial diagnosis is about six months. Sometimes, these esthesioneuroblastoma symptoms are misinterpreted and considered as a common condition. The various signs and symptoms are classified as following:

  • Nasal symptoms: Nasal obstruction, discharge, nose bleeding, sinus infections, and changes in the sense of smell
  • Oral symptoms: Ulcers in the mouth, loose teeth, and delayed healing after tooth extraction
  • Facial symptoms: Pain, swelling, numbness, and spasm of jaw muscles
  • Ocular symptoms: Vision abnormalities and excess tearing from your eyes
  • Ear symptoms: Painful sensations in the ear

Causes

The causes of esthesioneuroblastoma are still unclear. There are no specific genetic causes identified. But, it is suspected that various factors such as environmental pollution and cigarette smoking may give rise to blastoma. Medical experts believe that these tumors originate from various parts that include autonomic ganglia of the nasal mucosa, ectodermal olfactory placode, sphenopalatine ganglion, and olfactory epithelium.

Staging of esthesioneuroblastoma

The esthesioneuroblastoma staging is based on the Kadish system. The Kadish system focuses on the extent or spread of cancer (metastasizing).

Kadish A: Cancer is confined only to the nasal cavity

Kadish B: Cancer extends to the paranasal sinuses.=

Kadish C: Cancer spreads beyond the nasal cavities and paranasal sinuses

Kadish D: Cancer extends beyond the lymph nodes or other parts of the body

Your doctor would perform a thorough physical examination of your eyes, throat, and nose. A complete nasal evaluation is also done if you have a unilateral nasal obstruction and frequent nose bleeding that lasts for more than 1 to 2 months. If the tumor is suspected, then your doctor might order the following diagnostic tests.

Endoscopic examination: The test involves inserting an endoscope which a thin, flexible tube with a tiny camera into your mouth or nose. This helps your doctor to check the signs of a tumor.

Intranasal biopsy: The biopsy would help to identify the tumor in early stages. A small tissue from your nose is removed and examined under the microscope.

Unilateral nasal abnormality is the key to suspect a possible neoplasm

Computed tomography scans: A CT scan provides a 3-dimensional view of your internal parts. It is beneficial for accurate staging of your tumors.
Magnetic resonance imaging (MRI): The MRI scan provides more precise images of soft tissue structure of your nose.

The esthesioneuroblastoma treatment depends on the severity or stage of your tumors. Your doctor might recommend any of the following treatment strategies.

Surgery: The surgical resection is considered as the treatment of choice for esthesioneuroblastoma. A craniofacial resection is a traditional approach and a gold standard for treating the olfactory neuroblastoma. During the procedure, your surgeon makes an incision in the skull and along the sides of your nose; then, removes the tumor. Small tumors can be removed using a minimally invasive procedure called as trans-nasal endoscopic resection.
Radiation therapy: Often, the radiation therapy is accompanied by chemotherapy. The esthesioneuroblastoma radiology allows effective management of the olfactory neuroblastoma.
Chemotherapy: Your doctor may recommend esthesioneuroblastoma chemotherapy to reduce the size of the large tumor so that the tumor can be effectively removed through surgical resection.