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Esthesioneuroblastoma, also known as olfactory neuroblastoma (cancer that develops in immature nerve cells), is a rare type of cancer that develops in the upper portion of the nasal cavity. More specifically, it develops in the olfactory epithelium and nerve endings, which is near the bone separating the nasal cavity from the brain. Olfactory epithelium also plays a role in our sense of smell. Esthesioneuroblastoma is known to metastasise (spread to other parts of the body) to the sinus, eyes and brain; however, it can spread to other parts of the body as well. 

Esthesioneuroblastoma is most commonly diagnosed in adults and has higher incidence in males; however, anyone can develop this disease. Although it is rare for children and teenagers to develop this disease, esthesioneuroblastoma is the most common type of nasal tumour in these age groups.

Treatment

If an esthesioneuroblastoma is detected, it will be staged and graded based on size, metastasis and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

  • T (tumour) indicates the size and depth of the tumour. 
  • N (nodes) indicates whether the cancer has spread to nearby lymph nodes. 
  • M (metastasis) indicates whether the cancer has spread to other parts of the body. 

This system can also be used in combination with a numerical value, from stage 0 – IV: 

  • Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue. 
  • Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and has not spread to lymph nodes. This stage is also known as early-stage cancer. 
  • Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer. 
  • Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also classified as localised cancer. 
  • Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer. 

Cancers can also be graded based on the rate of growth and how likely they are to spread: 

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour. 
  • Grade II: cancer cells present as abnormal and grow faster than grade I cancers. This is also known as an intermediate-grade tumour. 
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour. 

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate course of treatment for you. 

Treatment is dependent on several factors, including location, stage of disease and overall health. There is no one treatment option for this type of cancer, due to the rarity of the disease.

Surgery

This procedure is generally used in patients whose esthesioneuroblastomas are low stage and grade, and who have a tumour that hasn’t metastasised (spread to other parts of the body). The procedure itself will vary depending on the location and size of the tumour, but usually involves removing the tumour and a margin of healthy tissue to help prevent recurrence. This can be done endoscopically, which uses a long, thin tube with a camera attached (endoscope) to remove affected portions of the nasal cavity, or via a procedure called a craniotomy, which involves removing a small portion of the skull to remove the tumour.

In many cases, surgery is used in conjunction with chemotherapy or radiation therapy. 

Radiation therapy

Radiation therapy, or radiotherapy, uses controlled doses of radiation to damage or kill cancer cells. It can be administered internally or externally. This may be a satisfactory alternative if you are not a suitable candidate for surgery. This treatment can also be used in addition to surgery. In some cases, radiotherapy is recommended prior to surgery to shrink the tumour so it’s easier to surgically remove. It can also be recommended after surgery to reduce the risk of cancer recurrence in the future.

Chemotherapy

Chemotherapy uses drugs to kill or slow the growth of cancerous cells, while minimising the damage to healthy cells. It is usually administered directly to the veins (intravenously), however in some cases in can be administered orally. You may receive a single chemotherapy drug, or a combination, depending on your individual factors. It is usually done in cycles of treatment sessions and periods of rest and can last several months. Chemotherapy can be recommended for patients prior to surgery in order to shrink the tumour, making it easier to surgically remove. In some cases, it can also be recommended after surgery to reduce the risk of cancer recurrence, or if your tumour is advanced and has metastasised. 

Clinical trials

Clinical trials are research studies performed to test new treatments. They present the opportunity for people, particularly those with rare or complex cancers, to receive very new treatments which are not yet available otherwise. While these studies have the potential to develop more treatment options, the risk of side effects can be high and are not always known. These trials might involve targeted therapies (to target only the cancer cells), hormonal therapies or immunotherapies.  

Clinical trials are often conducted in phases: 

  • Phase 0: these trials are used to determine how the body will react to experimental drugs. They are given in very small doses in very few people. 
  • Phase I: these trials are used in slightly bigger groups (20-80 people) to determine the safety of the drug, as well as any potential side effects that may arise. 
  • Phase II: these trials are used in larger groups (several hundred people) to further evaluate the safety of the drug, as well as whether it is working as it should be. 
  • Phase III: these trials are used in groups of several hundred to several thousand people. They are used to monitor any adverse effects of the drug, as well as compare it to other conventional or experimental treatment methods. 
  • Phase IV: these studies test the drug in the general population to determine any long-term adverse effects, as well as potential usage in treatment of other conditions. 

Ask your doctors about the availability of clinical trials, including whether you are suitable, and any benefits, risks and potential complications that may arise.  

Palliative care

Palliative care aims to improve the patient’s quality of life and alleviate symptoms, without trying to cure the disease. This option is beneficial for patients with advanced esthesioneuroblastomas at any stage of treatment, not just towards the end of life. It can also be used in conjunction with other treatment options, such as surgery, radiotherapy and chemotherapy, with the intention of relieving pain, alleviating symptoms and meeting your physical, emotional, cultural and spiritual needs.

Risk factors

Because of how rare esthesioneuroblastoma is, there has been limited research done into the risk factors of this disease.

In childhood cancers, it may be beneficial to ask your treating team about genome sequencing (a study to analyse your DNA) to better understand the contributors and genetic risk factors of this disease. 

Early symptoms

Symptoms of esthesioneuroblastomas will vary based on stage and metastasis. Early symptoms of this type of cancer include:

  • A mass in the nose.
  • Difficulty breathing through the nose.
  • Foul-smelling nasal discharge.
  • Frequent nosebleeds.
  • Eye bulges.

As the tumour progresses, other symptoms may appear, such as:

  • Eye and/or ear pain.
  • Headaches.
  • Loss of vision.

Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.

Diagnosis/diagnosing

If your doctor suspects you have an esthesioneuroblastoma, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment.

Physical examination

Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your eyes, nose, head and neck to check for any lumps or other abnormalities.

Imaging & blood tests

The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.

Endoscopy & biopsy

An endoscopy is a surgical procedure that involves inserting a long, flexible tube with a light and small camera through your nasal cavity and sinus area to observe any abnormalities. You will be given a sedative or anaesthetic throughout the procedure. You will be asked to fast for several hours prior to the procedure. An endoscopy is often done as a day surgery. Your doctor will discuss the risks and any possible complications prior to the procedure.

Throughout the procedure, your doctor may also perform an endoscopic ultrasound to guide the needle during a biopsy, or to check for signs of cancer metastasis. 

If any abnormalities are observed, your doctor will remove a small tissue sample for analysis.

Prognosis (Certain factors affect the prognosis and treatment options)

While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on rate and depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. Generally, early-stage esthesioneuroblastomas have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis. 

References

Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.