What is Esthesioneuroblastoma or Olfactory Neuroblastoma?

Esthesioneuroblastoma or Olfactory Neuroblastoma, is an extremely rare form of the Head and Neck Cancers. We can also call this cancer the Cancer of the Sense of Smell since it greatly affects the persons sense of smell and taste. One very important thing that has to be mentioned about esthesioneuroblastoma or olfactory neuroblastoma type of rare cancer is that it is not related at all to any other neuroblastoma malignant tumor found elsewhere in the body.

What is Esthesioneuroblastoma or Olfactory Neuroblastoma?

Causes of Esthesioneuroblastoma or Olfactory Neuroblastoma

You have probably concluded from the very fact that esthesioneuroblastoma or olfactory neuroblastoma is very rare and because of that its cause is unknown. If you had thought that way, you were right because the main cause of esthesioneuroblastoma or olfactory neuroblastoma is rather unknown. Moreover, the cause of this malignant tumor is one of the biggest medical mysteries.

It is so rare that the medical researchers and pathologists did not take up a lot of their time investigating the cause of esthesioneuroblastoma or olfactory neuroblastoma. However, there are certain possible causes of this cancer that were mentioned in the medical literature.

It is supposed that the main causes of esthesioneuroblastoma or olfactory neuroblastoma are genetic and environmental factors. People who had a family member with any Head and Neck cancer are at the greater risk of developing this malignant disease. Smokers are also at risk of developing Olfactory Neuroblastoma.

What confuses medical professionals is from which cells does esthesioneuroblastoma or olfactory neuroblastoma originate from. However, the most common cells whose abnormal growth causes olfactory neuroblastoma are the olfactory sensory cells which are found in the olfactory epithelium.

Symptoms of Esthesioneuroblastoma or Olfactory Neuroblastoma

As is the rule for all of the malignant diseases, the symptoms of esthesioneuroblastoma or olfactory neuroblastoma greatly depend on the extent and stage of that tumor. Sadly, this insidious disease does not show any signs and symptoms in its beginning stages.

The two tell-tale symptoms that might make your doctor suspicious of you having esthesioneuroblastoma or olfactory neuroblastoma are epistaxis and nasal obstruction. We can define nasal obstruction as the inability to breathe through each of the nostrils. Epistaxis is the medical term for nose bleeding. Bleeding very often from your nose and complaining about it to your medical doctor might have it think that you have esthesioneuroblastoma or olfactory neuroblastoma.

The most common symptoms of esthesioneuroblastoma or olfactory neuroblastoma are:

  • Nose bleeding.
  • Nasal obstruction.
  • Headaches.
  • Facial pain.
  • Proptosis (eyeball protrusion).
  • Double vision.
  • Ear pain.
  • Obstruction of the eustachian tube.

All of the symptoms above are related to the extension of the esthesioneuroblastoma or olfactory neuroblastoma tumor. Sadly, most of the symptoms of the underlying neuroblastoma resemble benign inflammatory processes and thus an enormous delay is often made with diagnosing this tumor. In most of the medical studies, the average time from the onset of symptoms to diagnosing esthesioneuroblastoma or olfactory neuroblastoma is six to twelve months. Because of this fact, most of the patients with this cancer are diagnosed when their tumor had invaded the surrounding organs and had entered the lymphatic system.

Tests to Diagnose Esthesioneuroblastoma or Olfactory Neuroblastoma

If your family physician suspects that you might have esthesioneuroblastoma or olfactory neuroblastoma, he/she would most probably send you to an ENT specialist. If any ENT specialist you were referred to suspects that you might have the rare cancer esthesioneuroblastoma or olfactory neuroblastoma, he/she will first order nasal endoscopy.

Nasal endoscopy is an invasive medical procedure. A small tube with a camera on its top will be inserted in your nose. The person who will perform this procedure will insert the tube with camera all the way through your nose to your sinuses. If this procedure confirms the presence of any lesion within your upper nasal cavity that resembles esthesioneuroblastoma or olfactory neuroblastoma, a procedure called intranasal biopsy will be ordered. If the biopsy results test positive for Esthesioneuroblastoma, two radiological tests will be ordered which are CT Scan and MRI scan.

A CT scan is needed to evaluate at what stage esthesioneuroblastoma or olfactory neuroblastoma is at. Also, CT scan shows the signs of cribriform plate erosion or skull base bone erosion which are both cause by esthesioneuroblastoma or olfactory neuroblastoma.

MRI scan is needed to better delineate intracranial, intraorbital, and sinonasal extension of esthesioneuroblastoma or olfactory neuroblastoma. Also, patients diagnosed with esthesioneuroblastoma or olfactory neuroblastoma should visit ophthalmologist since this condition usually causes minor or major damage to the eyes.

Staging of Esthesioneuroblastoma or Olfactory Neuroblastoma

There is no official staging system for esthesioneuroblastoma or olfactory neuroblastoma. However, the Kadish clinical staging system is used to describe the staging. According to Kadish clinical staging system, there are three stages of esthesioneuroblastoma or olfactory neuroblastoma which are:

  • Stage A
  • Stage B
  • Stage C

Stage A esthesioneuroblastoma or olfactory neuroblastoma is typically confined to the nasal cavity. Stage B esthesioneuroblastoma had extended to the paranasal sinuses. Stage C of ENB is described as a tumor that had extended the paranasal sinuses.

Treatment for Esthesioneuroblastoma or Olfactory Neuroblastoma

The surgery of esthesioneuroblastoma or olfactory neuroblastoma is a preferred treatment option for treating this malignant disease. After the successful surgery of esthesioneuroblastoma or olfactory neuroblastoma, a radiation therapy is performed. Radiation therapies used for treating this disease are Gamma Knife surgery and IMRT (Intensity Modulated Radiation Therapy).

For patients with a Kadish Stage C esthesioneuroblastoma or olfactory neuroblastoma, chemotherapy is prescribed. Doxorubicin, Cyclophosphamide, Vincristine, Etoposide, and Cisplatin are the chemotherapeutical agents used for the treatment.

Survival Rate for Esthesioneuroblastoma or Olfactory Neuroblastoma

The survival rate for esthesioneuroblastoma or olfactory neuroblastoma greatly depends on the stage this malignant disease had reached. These are the 5-year survival rates for the patients diagnosed with esthesioneuroblastoma or olfactory neuroblastoma according to this disease's stage:

  • Stage A - 95%
  • Stage B - 71%
  • Stage C - 47%.

Recovery Period/Healing Time for Esthesioneuroblastoma or Olfactory Neuroblastoma

Patients who had surgery of their esthesioneuroblastoma or olfactory neuroblastoma cancer usually stay at the hospital several days after the surgery. However, these patients will most likely lose their sense of smell.

Also, at least for several weeks, the patients who had esthesioneuroblastoma or olfactory neuroblastoma surgery would have to breathe through their mouth. Your doctor will provide the recovery period/healing time based on your particular case.

Prevention of Esthesioneuroblastoma or Olfactory Neuroblastoma

Although no prevention method regarding any cancer has been proved to greatly prevent it from developing, the best prevention method in terms of preventing esthesioneuroblastoma or olfactory neuroblastoma is quitting smoking.

Annual visits to the ENT specials are also the best way to diagnose early or prevent the development of esthesioneuroblastoma or olfactory neuroblastoma.

Risk Factors for Esthesioneuroblastoma or Olfactory Neuroblastoma

The risk factors for developing esthesioneuroblastoma or olfactory neuroblastoma are either genetic or environmental. The genetic risk factor for developing the malignant esthesioneuroblastoma or olfactory neuroblastoma is a family history or any other Neuroblastoma.

The environmental risk factors for developing esthesioneuroblastoma or olfactory neuroblastoma is being exposed constantly to polluted air and high doses of radiation. Also, smoking for a long period of time also poses a risk for developing this condition.

Prognosis/Outlook for Esthesioneuroblastoma or Olfactory Neuroblastoma

Luckily, esthesioneuroblastoma or olfactory neuroblastoma tumors are less aggressive than the other types of paranasal sinus carcinomas and sinus carcinomas. However, if this type of cancer is not diagnosed at its early stages, it spreads to the other parts of the body such as the brain and neck. Then the prognosis of esthesioneuroblastoma or olfactory neuroblastoma is fatal.

Coping with Esthesioneuroblastoma or Olfactory Neuroblastoma

The support of the family of a patient diagnosed with esthesioneuroblastoma or olfactory neuroblastoma is the best coping method for that patient. Patients diagnosed with the early and curable stages of this disease should be admitted to a psychologist for treating the initial shock of hearing they have cancer. Palliative care is the recommended coping method for the patients with esthesioneuroblastoma or olfactory neuroblastoma in terminal stages.

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Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: July 10, 2017

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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