Neuroblastoma

Neuroblastoma

NEUROBLASTOMA IN CHILDREN

Fewer than 100 children in the UK are diagnosed each year with neuroblastoma. Most children who get this cancer are younger than five years old. Neuroblastoma is the second most common solid tumour in childhood, and it makes up 8% of the total number of children’s cancers.

More children than ever are surviving childhood cancer. There are new and better drugs and treatments. But it remains devastating to hear that your child has cancer, and at times it can feel overwhelming. There are many healthcare professionals and support organisations to help you through this difficult time.

Understanding more about the cancer your child has and the treatments that may be used can often help parents to cope. Your child’s specialist will give you more detailed information. If you have any questions it’s important to ask the specialist doctor or nurse who knows your child’s individual situation.

You may find it helpful to read this information alongside our section on children’s cancers, which contains more information about cancers in children, their diagnosis and treatment and the support services available.

We hope this information answers your questions. If you have any further questions, you can ask a nurse or doctor involved in your child’s treatment.

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 MOST COMMONLY OCCURS IN:

either one of the two adrenal glands situated in the abdomen (tummy)
nerve tissue that runs alongside the spinal cord, in the neck, chest, abdomen
or pelvis.
The adrenal glands are specialised glands that are found above both kidneys. They normally release hormones to maintain blood pressure and help the body respond to stress.
In some cases, neuroblastoma can spread to tissues beyond the original site such as the bone marrow, bone, lymph nodes, liver and skin.
As with most cancers, the cause of neuroblastoma is unknown. It’s not infectious and can’t be passed on to other people.
Signs and symptoms of neuroblastoma Back to top
The symptoms vary depending on where your child’s tumour is. The first symptoms are vague, such as tiredness, loss of appetite and pain in the bones.

More specific symptoms will depend on where the neuroblastoma starts:
If the tumour is in the abdomen, your child’s tummy may be swollen and they may complain of constipation or have difficulty passing urine.
If the tumour affects the chest area, your child may be breathless and have difficulty swallowing.
If the tumour occurs in the neck, it’s often visible as a lump and occasionally affects breathing and swallowing.
Occasionally, there are deposits of neuroblastoma in the skin that appear as small, blue-coloured lumps.
If the tumour is pressing on the spinal cord, children may have weakness in the legs and walk unsteadily. If your child is not yet walking, you may notice reduced leg movements. They may also have constipation or difficulty passing urine.
Very rarely, children may have jerky eye and muscle movements, and general unsteadiness associated with the neuroblastoma.
Your child may also have high blood pressure.
A variety of tests and investigations may be needed to diagnose neuroblastoma.

These include a biopsy, blood and bone marrow tests, x-rays, CT or MRI scans, and special nuclear medicine scans called MIBG scans (see below). The tests are done to find out if your child definitely has neuroblastoma and what the exact position of the original tumour site is within the body. The tests will also find out whether the neuroblastoma has spread. This process is known as staging

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 the doctors to decide on the best treatment for your child.

A commonly used staging system for neuroblastoma is described below.

STAGE 1

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 after surgery.

STAGE 2A

The cancer is localised and has not begun to spread, but it cannot be completely removed by surgery.

STAGE 2B

The cancer is localised and has begun to spread into nearby lymph nodes.

STAGE 3

The cancer has spread into surrounding organs and structures, but has not spread to distant areas of the body.

STAGE 4

The cancer has spread to distant lymph nodes, bone, bone marrow, the liver, the skin or other organs.

STAGE 4S (ALSO CALLED SPECIAL NEUROBLASTOMA)

This is found in children under one year old. The cancer is localised (as in stage 1, 2A or 2B) but 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 a neuroblastoma tumour before treatment. These risk factors can be detected by scans and help doctors 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 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.

 

SURGERY

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 any 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.

 

CHEMOTHERAPY

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 a drip or injection into a vein. Your child’s specialist will discuss with you the type and amount of chemotherapy needed.

High-dose chemotherapy with stem cell support

If the neuroblastoma has spread to several parts of the body, or is high-risk with MYCN amplification, high-dose chemotherapy with stem cell support 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.

 

MONOCLONAL ANTIBODY TREATMENT

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 in people with high-risk neuroblastoma. Children in the UK with high-risk neuroblastoma are being given anti-GD2 as part of a clinical trial. There is good evidence from a clinical trial carried out in America in 2009 that this may be a promising therapy when given alongside other standard treatment for neuroblastoma.

It is not yet a standard treatment for people with neuroblastoma because it has very unpleasant side effects. The benefits still need to be fully proven and the best way to administer it needs to be confirmed. 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.

 

RADIOTHERAPY

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 (see above), but uses higher doses of radioactivity to kill the cancer cells.

 

YOUNGER CHILDREN

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 the tumour is causing symptoms. 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 on the actual treatment being given and the part of the body that is being treated. Side effects can include:

feeling sick (nausea) and being sick (vomiting)
diarrhoea
hair loss
increased risk of infection
bruising and bleeding
tiredness.
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 section on 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. Specialist doctors organise many trials for children’s cancers.

(Source Macmillan)



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