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Fewer than 100 children are diagnosed with neuroblastoma in the UK each year. Most children who get this cancer are younger than five years old.
Neuroblastoma is a cancer of specialised nerve cells, called neural crest cells. These cells are involved in the development of the nervous system and other tissues.
Neuroblastoma can occur anywhere in the body, but most often occurs in the adrenal gland in the abdomen (tummy). The adrenal glands are specialised glands which are found above the kidneys. They release hormones to maintain blood pressure, and enable us to respond to stress.
In some children, the neuroblastoma can occur in nerve tissue in the neck, chest, or around the spinal cord.
As with most cancers, the cause of neuroblastoma is unknown. As with other cancers, it is not infectious and cannot be passed on to other people.
The first symptoms are often vague, such as loss of appetite, tiredness and pain in the bones. Other symptoms vary, depending on where the neuroblastoma starts:
A variety of tests and investigations may be needed to diagnose a neuroblastoma. Tests such as x-rays, CT or MRI scans, bone marrow tests and blood tests are carried out to find the exact position of the neuroblastoma within the body and to see whether it has spread. This is known as staging.
A specific type of urine test will also be done. Nearly all (9 out of 10) children with neuroblastoma will have substances called vanyllylmandelic acid (VMA), or homovanillic acid (HVA), in their urine. Measuring the VMA and HVA in the urine can help to confirm the diagnosis. The child will also have their VMA and HVA levels checked during treatment. The levels of these substances will fall if the treatment is working. As these chemicals are produced by the tumour cells, and can be used to measure tumour activity, they are sometimes known as tumour markers.
An mIBG (meta-iodo-benzyl guanidine) scan may also be done. mIBG is a substance that is taken up by neuroblastoma cells. It is given by injection. Attaching a small amount of radioactive iodine to the mIBG enables the tumours to be seen by a radiation scanner. mIBG may also be used as a treatment.
A small sample of cells are usually taken from the tumour (biopsy) during an operation under a general anaesthetic. The cells are then examined under a microscope. Other tests, collectively referred to as tumour biology are also carried out on these cells in the laboratory.
The 'stage' of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type, and the stage, of the cancer helps the doctors to decide on the best treatment for the child.
A commonly used staging system for neuroblastoma is described below:
If the cancer has spread to distant parts of the body, this is known as secondary, or metastatic cancer.
If the cancer comes back after initial treatment, this is known as recurrent cancer.
The treatment of neuroblastoma depends on the age of the child, the size and position of the tumour, the tumour biology, and whether the neuroblastoma has spread.
For tumours that have not spread (localised tumours), the treatment is usually surgery. If the tumour is at an early stage and there is no evidence that it has spread to the lymph nodes or other parts of the body, an operation to remove the tumour, or as much of it as possible, will be done.In children with localised tumours, a cure is usually possible. However, if, due to the tumour biology results, the tumour is classed as 'high risk', further treatment will be needed. If the tumour is initially too large to remove safely, then chemotherapy will be given to shrink it down before surgery.
If the tumour has already spread by the time of diagnosis, or is indicated as being high risk by the tumour biology result, intensive chemotherapy is needed. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is usually given as injections and drips (infusions) into a vein. The child's specialist will discuss with you the type and amount of chemotherapy needed.
If the neuroblastoma has spread to several parts of the body, or is high risk, high-dose chemotherapy with stem cell rescue is used.
High doses of chemotherapy 'wipe out' the body's bone marrow (where blood cells are made). To prevent the problems that this causes, stem cells (blood cells at their earliest stages of development) are collected from the child through a drip, before the chemotherapy is given. They are then frozen and stored. After the chemotherapy, the stem cells are given back to the child (again through a drip). The stem cells make their way into the bone marrow where they grow and develop into mature blood cells.
If the neuroblastoma has spread to several parts of the body or is high risk, external radiotherapy may be given. This uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. External radiotherapy is given from a machine outside of the body. Internal radiotherapy may sometimes be given using radioactive mIBG. Radioactive mIBG is similar to the investigation used to diagnose a neuroblastoma, but uses higher doses of radioactivity to kill the cancer cells.