Neuroblastoma

A neuroblastoma is a malignant (cancerous) tumour, which develops in the nerve cells that run in a chain down the back of the chest and abdomen (the sympathetic chain).

In this section

The commonest place for this tumour to develop is in a gland just above one of the kidneys called the adrenal gland, but it can occur anywhere along the sympathetic chain.

Who gets neuroblastoma?

Less than 100 children develop neuroblastoma in the UK each year. It most often affects children under the age of five and is slightly more common in boys than girls. We do not know what causes neuroblastoma. There is no genetic tendency, nor is there any increased risk in their brothers or sisters.

Signs and symptoms

Neuroblastoma can present in many different ways, but usually it presents as a lump in the tummy (abdomen). Other symptoms include:

  • poor appetite
  • weight loss
  • lethargy
  • constipation
  • difficulty passing urine
  • bruising around the eyes
  • breathlessness
  • leg weakness (this is less common).

Tests / investigations

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. This will help us to decide on the best treatment for your child. These tests include:

  • Urine and blood samples – these will tell us how well the kidneys are working as well as some other information about general health. A specific test on the urine will be carried out to measure substances produced by the tumour. These are called ‘tumour markers’. In neuroblastoma two tumour markers are produced: vanillylmandelic acid (VMA) and homovanillic acid (HVA). Levels of these are often elevated at diagnosis.
  • Chest X-ray – this will tell us if there is tumour in the chest.
  • Ultrasound scan – this will tell us the position and size of the tumour. We can also use ultrasound scans to monitor the response to treatment.
  • CT scan – a scan of your child’s abdomen and lungs will be carried out. This will give us more detailed information about the tumour and may tell us if it has spread.
  • Biopsy – this is a small operation, usually carried out under general anaesthetic. The surgeon will remove a small piece of tissue from the lump, which will help us to make a diagnosis.
  • MIBG scan – this looks at places to which the tumour might have spread, such as bone, bone marrow and glands (lymph nodes). A mildly radioactive dye is injected which is taken up by neuroblastoma cells, and the scan is carried out the following day. This is quite harmless as the amount of radioactivity is very small. Your child lies on a couch with the camera above him/her and the scan takes about half-an-hour.
  • Bone marrow aspirate and trephine – as neuroblastoma may affect the bone marrow this is examined before treatment starts and possibly at stages during therapy.
  • Bone scan – this may tell us if tumour has spread to the bones. A mildly radioactive dye is injected on the day of the scan. This is quite harmless, as the amount of radioactivity is very small. A few hours later the scan is done. Your child will lie on a couch with the camera moving over him/her. The scan takes about half-an-hour.

Staging

Staging is a measure of how far the tumour has spread beyond its original site. The following describes the staging system we use for neuroblastoma:

  • Stage I: The tumour is localised with or without microscopic residual tumour after surgery.
  • Stage IIA: The tumour is localised but cannot be completely removed by surgery.
  • Stage IIB: The tumour is localised but has spread to local lymph nodes.
  • Stage III: The tumour crosses the midline, with or without local lymph nodes and cannot be removed surgically. There is localised tumour with lymph node involvement on the opposite side. There is midline tumour with lymph nodes on both sides.
  • Stage IV: The tumour has spread to distant lymph nodes, bone marrow, bone, liver and/or other organs.
  • Stage IVS: The tumour is localised (stage I, IIA or IIB), with spread limited to liver, skin and/or bone marrow in an infant under one year of age.

Treatment of Neuroblastoma

Treatment for neuroblastoma depends on the age, size and position of the tumour and whether it has spread. The treatment options include surgery, chemotherapy or radiotherapy, or a combination of these.

Surgery

Children with stage I and II tumours which can be completely removed will be treated with surgery alone providing the tumour has favourable features on biopsy. Where the tumour cannot be completely removed initially or has spread to other sites then the tumour will be removed after chemotherapy.

Chemotherapy

Treatment with anticancer drugs is used to destroy the cancer cells. It is usually given by injections and drips into a vein (intravenous infusion).

The type of chemotherapy will vary in intensity and duration according to the stage of the tumour, special features of the tumour (tumour biology) and response. Some children will need high-dose chemotherapy with a stem cell transplant.

If your child takes part in a clinical trial, the treatment is explained in more detail in the specific trial information sheet. You will be given an outline of the treatment in the form of a ‘road map’.

Radiotherapy

Radiotherapy may be used after surgery or high dose chemotherapy to destroy any remaining tumour cells. See the Children’s Unit booklet Radiotherapy treatment for more details on this type of treatment.

Clinical trials

Clinical trials are medical research trials involving patients that are carried out to try and find new and better treatments. In cancer, clinical trials are most commonly used to try and improve different forms of treatment such as surgery, radiotherapy or chemotherapy.

The treatment being tested may be aimed at:

  • improving the number of people cured (for example: trying new types of surgery or chemotherapy)
  • improving survival
  • relieving the symptoms of the cancer
  • relieving the side effects of treatment
  • improving the quality of life or sense of well-being for people with cancer.

Clinical trials may also involve research aimed at understanding more about the tumour’s biology. You may be asked to allow us to do research on the tumour sample removed at surgery, or on blood samples.

Well-run clinical trials have led to a significant improvement in the treatment of children with cancer. If you are asked to consider entering your child into a clinical trial you will be given specific information about the trial before deciding whether to take part.

Effects of treatment

The prognosis for neuroblastoma depends upon the stage of the tumour and its biology.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These may include growth problems, reduced fertility, hearing problems, impaired heart and kidney function and a small risk of developing a second cancer later in life.

About five years after treatment finishes we will transfer your child’s care to our long-term follow-up clinic. Your child will be seen at regular intervals in this clinic, indefinitely, so that we can help with any long-term effects of the treatment.