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). 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
- difficulty passing urine
- bruising around the eyes
- 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 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.