Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare tumor that occurs in the nerves of the nose responsible for the sense of smell. This condition often requires craniofacial resection surgery to remove the tumor. The use of radiotherapy or chemotherapy is limited for treatment due to the close proximity to the brain.
On this page:
What Is Olfactory Neuroblastoma?
Olfactory neuroblastoma is a rare cancer that develops in the upper part of the nasal cavity. It is thought to arise from neural tissue associated with the sense of smell.
Olfactory neuroblastomas generally grow slowly, but in some cases, they may progress rapidly and aggressively, nearly doubling their original size. The faster-growing tumors are capable of widespread metastasis.
How common is olfactory neuroblastoma?
Olfactory neuroblastoma is a very rare condition, accounting for about 3% to 6% of all tumors occurring in the sinonasal tract. The condition has only been seen about 1,000 times since its initial description in 1924.
The majority of observed cases were Caucasian men, with a median age of approximately 50. The severity of the condition was noticeably worse in the lower socioeconomic statuses, with a higher rate of mortality as well.
What causes olfactory neuroblastoma?
The direct cause of olfactory neuroblastoma is still unknown.
What are olfactory neuroblastoma risk factors and complications?
Any untreated tumor can create complications as the growth begins to apply pressure on the surrounding organs and musculoskeletal structures. Untreated tumors also run the risk of spreading to other, more vital organs.
Olfactory neuroblastoma risk factors
As there is no clear cause of olfactory neuroblastoma, there is little understanding of what risk factors make someone more susceptible to the condition.
Complications of olfactory neuroblastoma
Left undiagnosed or untreated, an olfactory neuroblastoma can be fatal. The growth of a tumor in a region like the sinuses can impact the function of several important organs in the skull and impair the ability to see or eat.
What Are the Signs and Symptoms of Olfactory Neuroblastoma?
The most common symptom of olfactory neuroblastoma is chronic congestion on one side of the nose. This stuffiness may continue for months or even years before a diagnosis is made.
Other symptoms may include:
- Loss of the sense of smell.
- Nasal discharge.
- Sinusitis.
Depending upon the size and extent of spread of the tumor, you may also experience:
- Facial swelling.
- Neurologic symptoms.
- Oral and vision problems.
Your doctor may refer you to a neurologist or an ophthalmologist to address these concerns.
When should I see a doctor about my olfactory neuroblastoma symptoms?
If you are experiencing symptoms like facial swelling or complications with the use of your mouth or eyes, you should see your doctor as soon as possible. The earlier cancer is caught and understood, the sooner your doctors can create a treatment plan. Olfactory neuroblastomas can also metastasize and spread to other local organs of the head and neck, creating further complications and difficulty of removal.
How Do You Diagnose Olfactory Neuroblastoma?
To diagnose olfactory neuroblastoma, your doctor will:
- Ask you about any symptoms you are experiencing.
- Perform a physical exam.
- Request imaging tests, such as MRI and CT scans, to confirm the tumor's presence
Tests to diagnose olfactory neuroblastoma
How Do You Treat Olfactory Neuroblastoma?
The primary treatment for olfactory neuroblastoma is commonly surgery, as the proximity to vital organs, like the brain, can create complications from chemotherapy or radiotherapy.
Surgery was commonly a craniofacial resection or transfacial surgery, where surgeons enter the nasal cavity and remove the tumor growths. However, recent developments have made minimally invasive surgeries more viable as they leave no incisions to heal and have a faster recovery time.
Minimally invasive surgery
At UPMC, the preferred minimally invasive surgical treatment for olfactory neuroblastomas is the endoscopic endonasal approach (EEA). This innovative, minimally invasive technique, pioneered and refined at UPMC, uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors.
Benefits of EEA include:
- Faster recovery time.
- No disfigurement.
- No incisions to heal.
Postoperative radiation therapy has been shown to lower the rate of recurrence.
Chemotherapy may be used in cases involving esthesioneuroblastomas that are recurrent, unresectable (not removable), or metastatic.
What happens during surgery?
Open surgery
As this is a rare cancer, there is no standard open surgery and the approach used will depend on the stage and size of the tumor.
In a standard resection, your surgeon will:
- Open up the area of operation with an incision (cut).
- Remove as much of the tumor as they safely can while avoiding vital regions and organs.
- Suture the skin closed with stitches.
In the endoscopic endonasal approach (EEA), your surgeon will:
- Enter the nose with an endoscopic device to view and complete surgery.
- Use specialized tools to break up the tumor.
- Use suction to remove fragments of the tumor until it is completely removed.
After surgery, radiation therapy may be used to eliminate remaining segments of the tumor.
How effective is treatment
Recurrence is common, unfortunately: Roughly 30% to 60% of patients who successfully had their initial tumor removed will have a recurrence. Treatment may require continued or additional chemotherapy or radiotherapy, as well as another surgery, if a large tumor develops again.
How long does it take to recover after surgery for olfactory neuroblastoma?
A traditional resection surgery can take several days in the hospital for immediate recovery and monitoring after surgery, but also more time for the sutures and stitches to heal.
An endoscopic endonasal surgery will not require as much time in the hospital as an open surgery, and can be completed more quickly and without incisions, making recovery easier. You still might need to stay in the hospital for monitoring and radiotherapy, should it be necessary.
