My son has just been diagnosed with neuroblastoma. Can you tell me what it is and what treatment he will have?
To answer your question, we have broken the information down into sections about
- What neuroblastoma is
- Causes of neuroblastoma
- Neuroblastoma symptoms
- Tests to diagnose neuroblastoma
- Stages of neuroblastoma
- Neuroblastoma treatment
Neuroblastoma is a cancer that affects children, mostly under the age of 5 years old. It is very rare. Only around 100 children are diagnosed with neuroblastoma each year in the UK.
Neuroblastoma develops from the cells left behind from a baby’s development in the womb. The cells that it develops from are called ‘neuroblasts’.
- ‘Neuro’ means nerve
- ‘Blast’ means cells in early development
- ‘Oma’ means a group of cells, or a tumour
Neuroblastoma often starts in the tummy (abdomen), commonly in the adrenal glands or the nerve tissue at the back of the abdomen. But it can spread to other parts of the body, such as the bones, liver and skin, through the blood and lymphatic system.
Neuroblastomas often produce substances called catecholamines (cat-a-coal-ay-meens). The adrenal glands make catecholamines, which are chemicals that send messages between nerve cells. Our bodies break them down into substances that we pass out in urine. In neuroblastoma the adrenal glands often produce catecholamines in larger amounts than normal.
We don’t know exactly what causes neuroblastoma. In some cases there is a family history of neuroblastoma. But these cases are very rare (1 in every 100 diagnosed).
The symptoms depend on where the cancer started in the body and whether it has spread to other parts of the body. Symptoms can be vague at first, such as tiredness, fever and loss of appetite. This can make neuroblastoma hard to diagnose in the early stages.
Because neuroblastoma usually develops in the abdomen, the most common symptom is a lump in the tummy. This may make the child’s tummy swell, causing discomfort or pain. Sometimes neuroblastoma can appear as a lump in the neck, perhaps causing breathlessness or difficulty in swallowing.
Neuroblastoma commonly spreads to the bones and can cause pain and difficulty in walking. Occasionally it can affect the spinal cord causing numbness, weakness and loss of movement in the lower part of the body.
If there is neuroblastoma in the bone marrow the child may have
- Anaemia due to a low red blood cell count
- Bruising or bleeding due to a low platelet count
- Infections due to a low white blood cell count
If neuroblastoma spreads to the skin it can give it a blue black colour, as if it were bruised.
Most children with neuroblastoma have higher than normal levels of catecholamines. The doctor will test for this, but it is very unusual for it to cause any symptoms. Very large amounts of catecholamines can cause symptoms such as
- Watery diarrhoea
- Weight loss
- Redness of the skin (flushing)
- A fast heart beat and increased blood pressure
Doctors carry out a number of tests if they suspect neuroblastoma. These aren’t generally painful. But your son will have to keep still for some of them. He is likely to need sedation or a general anaesthetic for some tests. He may have
- Blood tests
- Urine tests
- Chest X-ray
- CT scan
- MRI scan
- MIBG scan
- Bone scan
- A biopsy (taking a sample of tumour cells to examine in the lab)
- A bone marrow biopsy (a sample of cells from the bone marrow to see if there are neuroblastoma cells)
Doctors can use urine tests to help diagnose neuroblastoma. The waste products from catecholamines made by neuroblastoma cells are passed out of the body in the urine. Doctors can then test for these for chemicals, called HMA (homovanillic acid) and VMA (vanillylmandelic acid).
MIBG is a particular type of scan used to diagnose neuroblastoma. Neuroblastoma cells absorb a substance called MIBG. Doctors attach a very small amount of radioactive iodine to MIBG and then inject this into the bloodstream. The neuroblastoma cells pick up the MIBG and then the radioactive iodine attached to it shows up on the scan.
The stage of a cancer describes how far it has grown or spread. The stage is important because it helps the doctor decide which treatment to give. There are 4 main stages of neuroblastoma, but stages 2 and 4 are divided into two further groups.
Stage 1 means the tumour is in one area of the body and hasn’t spread anywhere. It can be completely removed with surgery.
Stage 2 is divided into 2A and 2B. In 2A, the tumour can’t be completely removed with surgery because of its size or position, but there are no cancer cells in any lymph nodes. In stage 2B, it may or may not be possible to completely remove the tumour, but it has spread to nearby lymph nodes.
Stage 3 - the tumour can't be completely removed with surgery. There is either neuroblastoma tumour on both sides of the body (either side of the spine) or there is tumour on one side of the body and lymph nodes containing cancer cells on the other.
Stage 4 - the cancer has spread to parts of the body that are some distance from where it started.
Stage 4S is a special case, as it has a better outlook than other stages. Stage 4S means the child is younger than 1 year old when diagnosed. Neuroblastoma may have spread to the liver or skin, but not to the bones. And no more than 10% of cells in the bone marrow are neuroblastoma cells.
As well as staging the neuroblastoma, doctors also group children into risk groups, according to their risk of the cancer coming back. Generally, the outlook is likely to be better if
- The child is younger
- The cells are more normally developed and so the cancer is likely to grow more slowly (this is called ‘low grade’)
- The neuroblastoma cells do not have a particular abnormal gene called MYCN
Your son’s treatment will depend on
- The stage, and
- The grade of the cancer
The grade means how close to normal the cells appear. The more normal they look under a microscope, the lower the grade and the more slowly the cancer is likely to develop.
Children with stage 4S neuroblastoma who have no symptoms may not need much treatment, or any treatment at all. This kind of neuroblastoma can disappear on its own. Doctors always keep a close eye on children who have had neuroblastoma, so they will have hospital appointments for some years.
Children with all other stages of neuroblastoma need to have treatment. They usually have a combination of
If possible, doctors use surgery to either remove the whole tumour, or as much of it as they can. The surgeon removes
- The tumour
- A border of tissue from around the tumour
- Any lymph nodes nearby
The exact type of surgery depends on where the tumour is in the body. If the tumour hasn’t spread, and it’s possible to remove it all, surgery may be the only treatment needed.
If neuroblastoma has spread to other parts of the body, the child is likely to need other treatment, such as chemotherapy.
Chemotherapy uses drugs to kill cancer cells. Your child may have chemotherapy
- Before surgery, to shrink the cancer and make it easier to remove
- If neuroblastoma has spread from where it started
- If tests show there is a high risk of the cancer coming back
Doctors usually give a combination of chemotherapy drugs, usually 2 or 3 different ones. The most common side effects of chemotherapy are
- Hair loss
- Feeling sick
- An increased risk of infection
- A sore mouth
Some children need more intensive chemotherapy. High doses of chemotherapy can damage the bone marrow and kill off the cells that make blood cells. So children who have high dose chemotherapy need further treatment afterwards to replace these cells, called blood stem cells. You can find more information in our section about stem cell transplants.
Radiotherapy uses high energy X-rays to kill cancer cells. You can have either
This means radiotherapy aimed at the cancer from outside the body. Doctors might give this radiotherapy before surgery to shrink the tumour, or after surgery to reduce the risk of the cancer coming back. Your son might have external beam radiotherapy on its own, or alongside chemotherapy. There is a picture of a radiotherapy machine below. It is important to keep very still during radiotherapy treatment. So small children sometimes have a short anaesthetic or sedative beforehand. There is information about the side effects of radiotherapy in our cancer treatments section.
Doctors use this type of treatment for some children with neuroblastoma that is advanced or has come back after earlier treatment. It is similar to the MIBG Scan used to diagnose neuroblastoma, but gives a higher dose of radioactive iodine. The MIBG is picked up by the cancer cells. The dose of radioactive iodine attached to the MIBG is high enough to kill the neuroblastoma cells, but not to cause serious side effects to surrounding tissues.
Of course it is extremely distressing to find out your child has cancer. It can raise many different emotions. You will probably feel angry, sad, upset and shocked. This is very normal. It is such a lot to take in. You are likely to worry about whether they will get the right treatment, what will happen, and how you will all cope.
As a parent you are probably worrying about any brothers and sisters as well. Practical help and support is something people nearly always need, for example, shopping or help with looking after other children in the family. It is essential that you also have support. Talking to friends and other members of the family can help you deal with your feelings. You can find out more about emotions and cancer in our coping with cancer section.
The Neuroblastoma Society is run by the parents and grandparents of children with neuroblastoma. They have a befriending scheme for affected families. You can find out about other organisations that offer help and support to children and their families on our general cancer organisations page.
The CCLG are the Children's Cancer and Leukaemia Group. They produce booklets for parents and brothers and sisters of children with cancer that you may find helpful. You can download these from their website. You can also find details of other helpful booklets and leaflets on our cancer reading list.
Many parents worry about the possible long term effects of treatment. Some children treated for neuroblastoma go on to develop long term side effects. But not everyone has them. The possible long term effects depend on
- The type of treatment the child has had
- The treatment doses they had
- The age they were when they had treatment
It is important to remember that the risk of these longer term effects is small. And the risk needs to be weighed up against the benefits of treatment. For many children, treatment will cure their cancer.
The risk of long term effects is also becoming less as treatments improve. So the treatment that children have now is less likely to cause long term problems than treatment in the past.
Possible long term effects include
- Fertility problems
- Hearing problems
- Changes in how the kidneys and heart work
- Growth problems
- Risk of developing a second cancer
Your child’s doctor will talk to you about the possible risks depending on the treatment your child has had. They will continue to keep a close eye on your child, so that any problems can be treated early.
In 2004, the Scottish Intercollegiate Guidelines Network (SIGN) produced guidelines about how children should be monitored after childhood cancer. These include recommendations about which professionals should be involved in following the child into their adult life. And in 2005 the Children’s Cancer and Leukaemia Group (CCLG) produced information to guide doctors on how best to do this.
Researchers are looking into the long term effects of treatment for all types of childhood cancer. The Centre for Childhood Survivor Studies is carrying out the British Childhood Cancer Survivor study. This research is looking at children who were diagnosed with cancer between 1940 and 1991 and lived for at least 5 years after diagnosis.
This type of research takes many years to produce results because the children need to be followed throughout their lives. So it will be some time before we know the results.
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