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This information is about neuroblastoma in children. You may find it helpful to read it alongside the Macmillan/CCLG booklet A parent’s guide to children’s cancer|, which contains more information about children's cancers, their diagnosis and treatment and support services.
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 that 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 (see below). 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 children with neuroblastoma (9 out of 10) 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's taken up by neuroblastoma cells. It's 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. These 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.
The presence of a certain amount of MYCN in the cells (known as MYCN amplification) can suggest that the disease may be more aggressive. In this situation, the treatment needs to be 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:
The cancer is contained within one area of the body (localised) and there's no evidence of it having spread. It can be completely removed by surgery|, or there may be very small (microscopic) amounts of tumour left.
The cancer is localised and has not begun to spread, but cannot be completely removed by surgery.
The cancer is localised and has begun to spread into nearby lymph nodes|.
The cancer has spread into surrounding organs and structures, but has not spread to distant areas of the body.
The cancer has spread to distant lymph nodes, bone, bone marrow, liver, skin or other organs.
This is found in children under one year old. The cancer is localised (as in stage 1, 2A or 2B) and has begun to spread to the liver, skin or bone marrow.
A newer staging system has been 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 in neuroblastoma tumours. These risk factors can be detected by scans and help doctors understand the extent of the disease. Your doctor can explain more about this.
The tumour is localised and has not spread into important areas (vital structures) nearby. It can be removed by surgery.
The tumour is localised but has 'image-defined risk factors' and can't be safely removed by surgery.
The tumour has spread to other parts of the body.
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 or relapsed 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's 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.
A cure is usually possible for children with localised tumours. However, if the tumour is classed as 'high risk' due to the tumour biology results, further treatment with chemotherapy and possibly radiotherapy will be needed. If the tumour is, at first, too large or in too difficult a position to remove safely, 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's 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 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 antibodies| 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. A report from a clinical trial (research study) carried out in America in 2009 suggests this may be a promising therapy when given alongside other standard treatment for neuroblastoma.
However, anti-GD2 has unpleasant side effects. It is not yet a standard treatment for people with neuroblastoma and is currently only available to patients in the UK who are being treated within a clinical trial. Patients with high-risk neuroblastoma may be able to receive anti-GD2 in the UK if they are being treated within the European high-risk clinical trial.
Your child's specialist will be able to tell you more about this treatment.
External radiotherapy| may be given if the neuroblastoma is high risk or if it 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 age 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.
Your child's doctor or nurse will talk to you about any possible late side effects. There's more detailed information about these late side effects in the booklet A parent’s guide to children’s cancer.
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. Specialist doctors organise many trials for children's cancer.
Children in the UK with neuroblastoma can take part in national and European studies. The European research group is called SIOPEN.
If appropriate, your child's medical team will talk to you about taking part in a clinical trial, and will answer any questions you have. Written information is 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's 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's 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.
It's not possible to address all of the feelings you may have here. However, the booklet A parent’s guide to children’s cancer talks about the emotional impact of caring for a child with cancer and suggests sources of help and support.
Your child may have a variety of powerful emotions| throughout their experience of cancer. The parent's guide discusses these 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 explore their feelings.
CCLG| coordinates 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 (there's a map of centres| on the website). Has information about the CCLG, childhood cancer and leukaemia.
CLIC Sargent| offers practical support to children and young people aged 21 and under with cancer or leukaemia, and to their families.
This section has been compiled using information from a number of reliable sources, including:
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.