Esthesioneuroblastoma, also known as olfactory neuroblastoma (cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that develops in immature nerve cellsthe basic structural and functional unit of all living things), 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 tumoura tissue mass that forms from groups of unhealthy cells in these age groups.
Treatment
If an esthesioneuroblastoma is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas 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 (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare esthesioneuroblastoma is, there has been limited research done into the riskthe possibility that something bad will happen 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.
Symptoms
Symptoms of esthesioneuroblastomas will vary based on stage and metastasis. Early symptoms of this type of cancer include:
- A massa growth of cells that come together to make a lump, may or may not be cancer in the nose.
- Difficulty breathing through the nose.
- Foul-smelling nasal discharge.
- Frequent nosebleeds.
- Proptosisbulging or protrusion of one or both of the eyes out of the eye sockets, also known as exophthalmos.
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
If your doctor suspects you have an esthesioneuroblastoma, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- Endoscopic ultrasoundan ultrasound taken during an endoscopy.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Endoscopya procedure that involves inserting a long, flexible tube with a light and small camera (endoscope) into the body to view internal organs, most commonly a nasoendoscopyexamination of the lining of the nasal passages and the nasopharynx with a small, flexible instrument known as a nasoendoscope.
- Biopsyremoval of a section of tissue to analyse for cancer cells.