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 tumours can occur anywhere in the body, but it most often occur in one of the adrenal glands in the abdomen (tummy). The adrenal glands are specialised glands that are found above the kidneys. They release hormones to maintain blood pressure, and enable us to respond to stress. In some children, neuroblastoma can occur in nerve tissue alongside the spinal cord in the neck, chest, abdomen or pelvis. It can occur at any age during childhood, but mainly occurs in young children less than five years old. The tumour is potentially fatal due to widespread secondary deposits that are normally present at diagnosis.
Medical text written November 2010 by Dr BL Pizer, Consultant Paediatric Oncologist, Alder Hey Children’s Hospital, Liverpool, UK.
Initial symptoms often include loss of appetite, tiredness and pain in the bones. Other symptoms vary, depending on where the child’s neuroblastoma starts:
- if the tumour is in the abdomen, the tummy may be swollen and there may constipation or difficulty passing urine. In some cases, the child’s blood pressure is high
- if the tumour affects the chest area, the child may be breathless and have difficulty swallowing
- if the tumour occurs in the neck, it is often visible as a lump and may affect 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 the child is not yet walking, reduced leg movements may be noted. The child 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.
Tumours occur when the genes in cells that instruct them to grow and multiply in an orderly and controlled way have a mutation. In these cancerous cells, the change in these instructions causes the cells multiply uncontrollably forming a tumour.
A variety of tests and investigations may be needed to diagnose a neuroblastoma. A urine test for vanillylmandelic acid (VMA), or homovanillic acid (HVA) can be carried out in children suspected of having a neuroblastoma as the presence of VMA or HVA is indicative of the disease. mIBG (meta-iodo-benzyl guanidine) scanning can be used to visualise tumours. mIBG is readily taken up by a neuroblastoma and when a small amount of radioactive iodine is attached it enables tumours to be seen by a radiation scanner. A biopsy (removal of a small section of the tumour for further study) will allow the biology of the tumour to be analysed.
The treatment of neuroblastoma depends on the age of the child, the size and position of the tumour and whether the neuroblastoma has spread from its original location.
Is used for tumours that have not spread (localised tumours) to remove the tumour. If the tumour is, at first, too large or in too difficult a position to remove safely, then chemotherapy will be given to shrink it before surgery.
Is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. A combination of different drugs is used. If the neuroblastoma has spread to several parts of the body, or is ‘high risk’ high dose chemotherapy may be used.
This uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells.
Retinoic acid therapy
This is given at the end of therapy for high-risk neuroblastoma. Retinoic acid is a drug similar to vitamin A that is given by mouth. It is a fairly safe drug but may cause skin rashes.
This uses antibodies to treat high-risk neuroblastoma. Antibodies are proteins that we all produce to fight infections. In this type of therapy, antibodies have been designed to attack neuroblastoma cells.
The Society is a Registered Charity in England and Wales No. 326385. It provides information and support to families with children affected by neuroblastoma, and gives them the opportunity to talk to other families. The Society raises funds for medical research into improving both diagnosis and treatment of the disease.
Group details last updated August 2014.