Frequently Asked Questions about Neuroblastoma
Just click on any of the links below to go directly to the answer for that question.
- What is neuroblastoma?
- What are the symptoms?
- What happens next?
- Diagnosis
- Neuroblastoma staging
- I can't remember what the doctor said
- Further information
What is neuroblastoma?
Neuroblastoma is a tumour arising from particular nerve cells, which run in a chain-like fashion up the back of the child's abdomen and chest and into the skull following the line of the spinal cord (neuro = nerve, blastoma = collection of tumour cells). The tumour occurs either in the sympathetic nervous system or closely associated adrenal glands and is usually, although not always, located in the abdomen.
Because the symptoms of the disease tend to be varied and vague, some two-thirds of children are not diagnosed until the disease is widespread. It is a tumour almost exclusively of childhood and there is no exactly comparable tumour in an adult.
It has its own specialist treatment that is different from any treatment undergone by anyone else you know who has had cancer. Despite intensive courses of chemotherapy and often intial success in treating the disease, the child often relapses, making this one of the most lethal of all childhood cancers.
What are the symptoms?
The site of the tumour may cause different symptoms at the time of diagnosis. For example, a tumour right at the back of the abdomen, which has grown to press on the spinal cord, may cause a variety of symptoms including an unsteady walk or difficulty passing urine. A child who has a tumour in the chest may have an initial problem with a chest infection, cough or fluid in the lung, which may need treatment before the neuroblastoma itself is tackled.
Many children have little in the way of symptoms. Perhaps they have seemed 'off-colour' for a little while or have a loss of apetite, vague aches and pains or sweating. Unless a parent or doctor feels a lump, while bathing, dressing or examining the child, a diagnosis of neuroblastoma may not be initially considered.
What happens next?
Once neuroblastoma is suspected, your child will be referred to a specialist child cancer treatment centre for diagnostic tests and a treatment plan. Although neuroblastoma is rare, these centres will see the majority of children who are diagnosed and thus they build up and maintain a body of knowledge and expertise about the tumour and its treatment. Such an approach would hardly be possible in a small hospital where neuroblastoma is never treated.
Diagnosis
The diagnosis of neuroblastoma is usually confirmed by tests at the specialist treatment centre. Tests your child will most probably undergo are clinical examination (the doctor's physical examination), blood and urine tests, special scans, X-rays and bone marrow tests, as well as a 'biopsy' of the main tumour mass. All of these may seem frightening to both you and your child, but the reason for having each test will be discussed in detail.
Neuroblastoma staging
Doctors recognise several special categories of neuroblastoma that are grouped into different 'Stages'. Staging means how far the tumour has spread in the body. The different stages of neuroblastoma have different treatments, as they carry with them different risks. Other factors that may affect your child's prognosis are their age and the results of several laboratory tests that will be carried out during the initial investigation period.
For many years the International Neuroblastoma Staging System (INSS) has been used to assess individual children and these descriptions are likely to continue for some time alongside a newly introduced International Neuroblastoma Risk Group Staging System (INRSS). The new system is aimed at getting a better match between the risks and side-effects of therapy (treatment) with the risk of disease. As these new staging definitions are adopted worldwide it will also be possible to get a more accurate comparison of the effectiveness of different treatment strategies. The approximate INRSS stage is shown in brackets against the INSS stage below. You can download more information about this new system here.
Stage 1 Neuroblastoma (INRSS stage L1)
This means that the tumour is fairly small and confined to one site. It has not spread anywhere else in the body and is able to be removed completely by an operation. This type of tumour is usually curable by surgery alone.
Stage 2 Neuroblastoma (INRSS stage L1)
As with Stage 1, the tumour is confined to one site and has not spread to distant parts of the body. However, it may be larger than a Stage 1 neuroblastoma and, at operation, it may be more difficult to remove completely. Sometimes, a lymph node or some glands near to the Stage 2 neuroblastoma may also have been affected by the tumour. Stage 2 neuroblastoma is usually treated by surgery alone but, depending on the site and results of certain tests, additional treatment such as chemotherapy may be needed.
Stage 3 Neuroblastoma (INRSS stage L2)
This stage of tumour is also confined to the primary site in that it has not spread to other distant parts of the body. The tumour may be very large and said to have crossed the 'mid-line' of the body. This means that the tumour has grown right across the child's abdomen or chest from the original side where it began. In the case of Stage 3 neuroblastoma, drug treatment will initially be recommended to try to shrink the tumour for subsequent surgical removal. After the operation, radiotherapy may be given.
Stage 4 Neuroblastoma (INRSS stage M)
This means that the primary tumour may be of any size, but some neuroblastoma cells have broken away and spread to other organs of the body, most commonly bones, bone marrow or liver. Drug therapy will be recommended for Stage 4 neuroblastoma. This has two aims:
(a) To kill the tumour cells that have spread to different body organs.
(b) To shrink the primary tumour for later removal by an operation.
After surgery, further drug therapy is given, using very large doses of chemotherapy. After this 'high dose chemotherapy', the next part of treatment may involve radiotherapy which is treatment using high strength X-rays. Finally fairly gentle treatment is given to mop up the few remaining tumour cells that may be left in the body.
Infants with Stage 4 Neuroblastoma (INRSS stage M)
Most infants under one year of age who present with Stage 4 neuroblastoma have less 'aggressive' neuroblastoma than that occurring in older children. Therefore, they may receive a less intensive course of chemotherapy than a toddler or older child.
Stage 4S Neuroblastoma (INRSS stage MS)
This is a special type of tumour found in very young babies, under one year old. The tumour cells may have spread beyond the 'primary' site to other parts of the body, but the cells usually behave in a less aggressive fashion than in the older child. The tumour may shrink and disappear without treatment. Alternatively, low doses of either radiotherapy or chemotherapy may be given to encourage the tumour to shrink. The sites affected by Stage 4S neuroblastoma are typically liver, skin and sometimes bone marrow. When this pattern is noted, the Oncologist may feel fairly confident that the child will get better with no treatment or very little treatment. Sometimes, some weeks or months after diagnosis, surgical removal of the 'primary' tumour will be recommended. Also, sometimes the tummy may swell so much with the tumour that chemotherapy is needed to shrink the tumour and relieve the pressure in the abdomen. Very occasionally, a baby with a Stage 4S neuroblastoma may later develop signs and symptoms of the more 'aggressive' Stage 4 neuroblastoma. The cells typically appear in the lymph glands or bones, and in this case the doctors may then decide to give drug treatment.
I can't remember what the doctor said
If you have been told that your child is suffering from neuroblastoma, it may be very difficult to grasp and remember what has been said as you may feel bewildered, frightened and shocked. It is probable that you have never heard the word 'neuroblastoma' before and may not have even realised that children suffer from tumours. Perhaps, like many people, your ideas about cancer have come from the media, or from older relatives in your family who may also have been treated for cancer. Cancer is a disease that leads to many 'old wives tales', and it is important that you try very hard to bear in mind the treatment team's assurance that many children's cancers, including neuroblastoma, differ a lot from adult tumours.
In recent days, you may have met a great many new faces, heard a lot of strange medical terms and your child may have undergone a series of puzzling and worrying investigations. All the hospital staff involved with your child understands that you may need some time to absorb what has happened, and what you are being told about their treatment. You must ask if you do not immediately understand something. You may need to ask some questions several times and, if so, don't feel that people imagine you are either foolish or unintelligent. At a time like this, it is very common to find it difficult to take in or remember information that you are given.
If the language used by the treatment team is not your first language, and you have difficulty in understanding the language used, arrange for a relative or friend to be present who can interpret. If you cannot find anyone to help, an interpreter can be arranged via the Social Work Department or through other contacts within the hospital.
Many parents cannot think of any questions to ask when a doctor is there, but think of all sorts of things as soon as they have gone. It is a good idea to write down things that you especially want to know, so that you can discuss your questions at the next opportunity. Some people are shy of questioning or approaching members of the treatment team, feeling they are too busy. They probably are very busy, but they are caring for your child and want to know of your concerns and discuss them with you.
If a particular moment is inconvenient, it may be suggested to you that later on the doctor and perhaps other member of the team could sit down with you and go over your concerns. This is not to ignore your urgent worry, but to provide a setting and a time when unhurried discussion can take place. It may be that the doctor has to wait for results of certain tests before any realistic discussion can take place, but it is understood that such a time of waiting is a very difficult and anxious one for you and your family.
Further information
The Society has produced a Booklet for Parents giving information about neuroblastoma and advice for parents on coping with a child who has the disease. Copies of this free booklet have been supplied to each specialist child cancer treatment centre so that every parent of a child with neuroblastoma can have one. If your treatment centre staff do not offer you a copy, please ask. Alternatively, a printed copy can be sent if you email your name and address to the secretary@neuroblastoma.org.uk, or to download a copy click here: Booklet for Parents. For this document you will need to have Adobe Acrobat Reader installed on your computer. You can download a free copy from www.adobe.com.
The Children's Cancer and Leukaemia Group (CCLG) provides a great deal of information for all members of the family affected by childhood cancers including neuroblastoma. Download a leaflet here for details of some of the resources available and how to obtain them. (Please note this is a very large file and may take several minutes to download depending on your connection speed). All publications listed are free to families and professionals in the UK.