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Macmillan and Cancerbackup merged in 2008. Together we provide free, high quality information for people affected by cancer through our publications, website and phone service. Find out more| .
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This information is about neuroblastoma in children. It is helpful to read it alongside the Macmillan/CCLG booklet A Parent’s Guide to Children’s Cancers| , which contains more information about cancers in children, their diagnosis and treatment, and the support services available.
Fewer than 100 children are diagnosed each year with neuroblastoma in the UK. Most children who get this cancer are younger than five years old. Neuroblastoma is the second most common solid tumour in childhood and makes up 8% of the total number of children's cancers.
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 it most often occurs in one of the adrenal glands 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 alongside the spinal cord in the neck, chest, abdomen or pelvis.
As with most cancers, the cause of neuroblastoma is unknown. It's not infectious and can't 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 your child's neuroblastoma starts:
A variety of tests and investigations may be needed to diagnose a neuroblastoma. These include: blood, urine, or bone marrow tests; x-rays, CT or MRI scans and mIBG scans. The tests are carried out to find the exact position of the neuroblastoma within the body and to see whether it has spread. This process is known as staging.
A specific type of urine test will also be done. Nearly all (nine out of 10) children with neuroblastoma will have substances called vanillylmandelic acid (VMA), or homovanillic acid (HVA), in their urine. Measuring the VMA and HVA in the urine can help to confirm the diagnosis. Your child will also have their VMA and HVA levels checked during and after 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.
Most children will have an mIBG (meta-iodo-benzyl guanidine) scan . 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 is usually taken from the tumour during an operation under a general anaesthetic. The cells are then examined under a microscope. This is called a biopsy. Other tests, collectively referred to as tumour biology are also carried out on these cells in the laboratory. These tests look at the chromosomes and 'biological markers' in the tumour cells. One of these markers' is called MYCN. If a certain amount of MYCN is present in the cells (known as MYCN amplification) this suggests the disease may be more aggressive. In this situation the treatment is more intensive.
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 stage of the cancer helps doctors to decide on the best treatment for your child.
A commonly-used staging system for neuroblastoma is described below.
A newer staging system is currently being developed by the International Neuroblastoma Risk Group (INRG). This system (described below) is beginning to be used more widely. It looks at whether or not certain 'image-defined risk-factors' are present. These risk factors can be detected by scans and help doctors to understand the extent of the disease. Your doctor can explain more about this.
Stage L1 The tumour is localised and has not spread into important areas (vital structures) nearby. It can be removed by surgery.
Stage L2 The tumour is localised but has 'image-defined risk factors' and can't be safely removed by surgery.
Stage M The tumour has spread to other parts of the body.
Stage MS The tumour has spread to the skin, liver and/or the bone marrow in children younger than 18 months old.
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 (including the MYCN status) 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 the tumour is classed as 'high-risk', due to the tumour biology results, further treatment will be needed. If the tumour is, at first, too large or in too difficult a position to remove safely, then chemotherapy will be given to shrink it 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. Your 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 with MYCN amplification, high‑dose chemotherapy with stem cell rescue| is used (after the initial courses of chemotherapy).
High doses of chemotherapy 'wipe out' any remaining neuroblastoma cells but they also 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 your child through a drip before the chemotherapy is given. These stem cells are then frozen and stored. After the chemotherapy, the stem cells are given back to your child through a drip. They make their way into the bone marrow, where they grow and develop into mature blood cells over a period of 14–21 days.
Monocolonal antiobodies can destroy some types of cancer cells while causing little harm to normal cells. A new monoclonal antibody treatment called anti-GD2 is currently being tested for people with high-risk neuroblastoma. Early reports from American research studies (2009) suggest this may be a promising therapy when given alongside other treatment. However, anti-GD2 has some unpleasant side effects and further studies need to be carried out before it can be considered a standard treatment. Anti-GD2 is not yet available in the UK, but this is currently under review. Your child's specialist will be able to tell you more.
External radiotherapy| may be given if the neuroblastoma is high risk, or has spread to several parts of the body. 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 the body.
Internal radiotherapy may sometimes be given using radioactive mIBG. Radioactive mIBG is similar to the mIBG used in an investigation to diagnose a neuroblastoma, but uses higher doses of radioactivity to kill the cancer cells.
Children under 18 months old with neuroblastoma often have 'low -risk' tumours, and as long as there is no MYCN amplification their outlook is good. Most children in this group are cured.
Children with Stage 4S disease almost always get better with very little treatment or none at all. These tumours either regress spontaneously or after chemotherapy (which is only given if there are symptoms from the tumour). They disappear completely or develop into a non-cancerous (benign) tumour, called a ganglioneuroma. Many of these children, after their initial diagnostic tests and staging investigations, will just need careful monitoring for some years.
Ganglioneuromas are usually harmless and will not cause any problems or need any treatment.
Treatment often causes side effects, and your child’s doctor will discuss this with you before the treatment starts. Any possible side effects will depend upon the actual treatment being given and the part of the body that is being treated. Side effects can include:
A small number of children may develop late side effects, sometimes many years after treatment. These include a change in the way the heart and kidneys work, hearing problems, fertility problems, a possible reduction in bone growth (if radiotherapy has been given) and a slightly increased risk of developing another cancer in later life. More detailed information about these long-term side effects| is available in the Macmillan/CCLG booklet, A Parent’s Guide to Children’s Cancers| .
Many children have their treatment as part of a clinical research trial| . Trials aim to improve our understanding of the best way to treat an illness, usually by comparing the standard treatment with a new or modified version of it. Specialist doctors carry out trials for children's cancer.
Many UK neuroblastoma trials now involve children from all over Europe. The European neuroblastoma group is called SIOPEN. If appropriate, our child's medical team will talk to you about taking part in a clinical trial, and will answer any questions you may have. Written information is often provided to help explain things.
Taking part in a research trial is completely voluntary, and you'll be given plenty of time to decide if it is right for your child.
Follow-up after treatment usually involves regular visits to the hospital outpatients department, with scans and urine tests as necessary. For children who have had chemotherapy and/or radiotherapy, more specialised tests – for example, hearing tests, kidney and heart function tests and checking hormone levels – may also be carried out. These will be repeated until your child is grown up.
If you have specific concerns about your child’s condition and treatment, it is best to discuss them with your child’s doctor, who knows their situation in detail.
As a parent, the fact that your child has cancer is one of the worst situations you can be faced with. You may have many different emotions| , such as fear, guilt, sadness, anger and uncertainty. These are all normal reactions, and are part of the process that many parents go through at such a difficult time. However, the booklet A Parent’s Guide to Children’s Cancers| talks about the emotional impact of caring for a child with cancer, and suggests sources of help and support.
Your child may have a range of powerful emotions throughout their experience of cancer. The Parent's| guide discusses this further and talks about how you can support your child. Our booklet Peppermint Ward| is a storybook for younger children with cancer. It looks at the issues they and their family may face and helps them to explore their feelings.
Neuroblastoma Society
Tel 020 8940 4353
Email secretary@neuroblastoma.co.uk|
www.nsoc.co.uk|
The society offers an opportunity for parents to give each other mutual help, support and comfort. There is an information booklet, which can be downloaded from the website.
CLIC Sargent
Griffin House, 161 Hammersmith Road, London W6 8SG
Tel 0800 197 0068
Email helpline@clicsargent.org.uk|
www.clicsargent.org.uk|
Offers practical support to children and young people aged 24 and under with cancer or leukaemia, and their families.
Children's Cancer and Leukaemia Group (CCLG)
University of Leicester, 3rd Floor, Hearts of Oak House, 9 Princess Road West,
Leicester LE1 6TH
Tel 0116 249 4460 (main office)
Email info@cclg.org.uk|
www.cclg.org.uk|
Co-ordinates research and care for children and their parents. There are 21 CCLG specialist centres for the treatment of childhood cancer and leukaemia, covering all areas of the UK and Ireland. A map of the centres is on the website – see above. Information about the CCLG and childhood cancer and leukaemia resources can be obtained from the address above.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography| .
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