The first symptoms of neuroblastoma are generally vague, such as loss of appetite, tiredness and pain in the bones.
Other symptoms vary, depending on where the child’s neuroblastoma starts:
- If it is in their abdomen, their tummy may be swollen and they may complain of constipation or difficulty passing urine
- If the tumour is in the chest or neck area, the child may be breathless and have difficulty swallowing
- Occasionally there may be deposits of neuroblastoma in the skin that appear as small, blue-coloured lumps
- If the tumour is pressing on the spinal cord, it may cause weakness in the legs.
A variety of tests and investigations may be needed to diagnose a neuroblastoma cancer. These include blood, urine or bone marrow tests, X-rays, CT or MRI scans and mIBG scans (see below). These tests confirm the diagnosis and help doctors to determine the best way to treat the tumour.
mIBG is a substance that is taken up by neuroblastoma cells. It is given by injection with a small amount of radioactive iodine attached to enable the tumours to be seen by a radiation scanner. mIBG may also be used to deliver treatment directly to the tumour.
Tumour cells are taken by biopsy to look at the chromosomes and ‘biological markers’ and help inform the type and intensity of treatment required. If a certain level of a marker called MYCN is detected it indicates that the tumour is high-risk and that treatment needs to be more intensive.
Staging of Neuroblastoma
The stage of a cancer describes how far it has grown or spread. The stage is important because it helps determine the best treatment. There are four main stages of neuroblastoma cancers:
Stage 1 means the tumour is in one area of the body and hasn’t spread. 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 means that the tumour can’t be completely removed with surgery and there is either 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 means that 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 one year at diagnosis. The tumour 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.
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 success, 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.
A newer staging system has been developed by the International Neuroblastoma Risk Group (INRG). This system 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.
Stage L1 – the tumour is localised and has not spread into 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.
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