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.
Treatment options for esthesioneuroblastoma may include:
- Surgery to remove as much of the tumour as possible.
- Radiation therapy.
- Chemotherapy.
- Clinical trials.
- Palliative care.
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
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.
Some of the information regarding symptoms was obtained from the Childhood Esthesioneuroblastoma Treatment (PDQ) page published by the National Cancer Institute.
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.