Neuroblastomas are neuroendocrine tumours that develop from neuroblast cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be cancer cells
(young nerve cells) in nerve tissue. Neuroblasts are most commonly found in the adrenal glands, which are located on top of the kidneys; however, they can also be found in the nerve tissues of the neck, chest, abdomenstomach, stomach area, belly, tummy or pelvis.
Neuroblastomas are a common type of neuroblastic tumour. Neuroblastic tumours are a group of tumours that arise from neuroblasts and/or paraganglia cells, which produce catecholamine hormones in nerve tissue. These tumours often develop within the sympathetic chain in the body, which consists of the bilateralBoth sides. nerves that run parallel to the spine from the base of the skull to the coccyx. Other common types of neuroblastic tumours include Ganglioneuroma and Ganglioneuroblastoma. For more information on neuroblastic tumours, please refer to the Rare Cancers Australia Neuroblastic Tumours page.
Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, bloodhello pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreas and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cells, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response.
Neuroblastomas are the most common type of solid childhood tumour and is usually diagnosed between birth and five years of age. However, it can be diagnosed in people at any age.
Treatment
If a neuroblastoma is detected, it will be staged and graded based on size, metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (whether the cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems. has spread to other parts of the body) 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 lymphA clear fluid that moves through the body through the lymphatic system, carrying cells that fight infection. 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 had 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 localisedOnly to one area/place 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 known 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:
- Grade
• A score that tells how quickly a tumour might spread and grow by looking at how the abnormal cells and tissue look under a microscope.
• Grade is not the same as stage.
• Grading is different depending on the type of cancer.
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 tumours. 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 treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatmento options for neuroblastoma may include:
- Surgery to remove as much of the tumour as possible.
- Radiation therapy.
- Chemotherapy
• a chemical drug treatment to kill or slow-growing cancer Cells
• these drugs are called cytotoxic drugs
. - Immunotherapy.
- Clinical trials, potentially including vaccine therapies.
- Palliative careLessening pain without curing the disease..
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
While the exact cause of neuroblastomas remains unknown, the following factors have been linked to a higher chance of developing the disease:
- Family history of neuroblastoma.
- Certain genetic conditions, such as Hirschsprung disease, congenital central hypoventilation syndrome (CCHS) and neurofibromatosis type 1 (NF1).
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
The symptoms of neuroblastomas will vary depending on stage, location and whether the tumour has metastasised (spread to other parts of the body). Some of these symptoms include:
- Lump or swelling in the affected area.
- Abdominal pain.
- Unexplained weight loss/loss of appetite.
- Issues urinating and/or with bowelThe intestines (like a tube) that run between the stomach and anus (bum) and is made up of the small bowel (small intestine) and the large bowel (colon and rectum). (see ‘small intestine’; and ‘colon’ for more information). movements.
- Exophthalmos (bulging eyes).
- Dark circles around the eyes.
- Uncontrolled eye movements.
- Bone and/or other unexplained pain.
- General weakness or paralysis.
- Difficulties breathing and/or swallowing.
In some cases, neuroblastomas release hormones that causes additional symptoms:
- DiarrhoeaWhere watery or loose faeces (stools; poo) is frequently/often released discharged from the body. Also called ‘the runs’..
- High blood pressure.
- Rapid heartbeat.
- Unexplainable sweating.
- Flushing of the skin.
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 a neuroblastoma, they will order a range of diagnostic tests.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), x-ray, bone scan, metaiodobenzylguanidine (MIBG) scan and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography scan.), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis. Additionally, a blood and urine test may also be taken to assessTo measure, look at and learn from. your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsyTo take a small piece of body tissue and test it in a laboratory. to remove a section of tissue using a needle. In most cases, this will be done by a bone marrowIs soft, spongy tissue found in bones that makes blood cells. aspiration and biopsy. This process involves inserting the needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow. These samples will then be analysed for cancer cells.