It is important to remember that every child is an individual and your child’s specific diagnosis must always be discussed with the treatment team caring for them.
We recommended reading our booklet, ‘A Parent’s Guide to Neuroblastoma’, which we hope helps answer your questions so that you understand more about neuroblastoma and its treatment.
What is neuroblastoma?
Neuroblastoma is a cancer that is almost always found in children. Childhood cancers are usually different from cancers affecting adults. They tend to happen in different parts of the body to adult cancers and behave differently. Cancers in children also respond differently to treatment.
Neuroblastoma is classified as an embryonal tumour, a type of cancer that develops from the cells left behind from a baby’s development in the womb. The cells from which it develops from are specific cells in the nervous system called neuroblasts, giving neuroblastoma its name:
‘neuro’ means nerve
‘blast’ means cells in an early stage of development
‘oma’ means a group of cells, or a tumour
The tumour occurs either in the sympathetic nervous system or closely associated adrenal glands and is usually, although not always, located in the abdomen.
Who is affected by neuroblastoma?
Neuroblastoma affects around 100 children each year in the UK, about 6% of the total number of childhood cancer diagnoses.
It usually affects children under the age of five, and can occur before a child is born. It is the second most common solid tumour in children after brain tumours.
What are the symptoms of neuroblastoma?
Many children have little in the way of symptoms and it is often hard to diagnose neuroblastoma in the early stages. Neuroblastoma, particularly ‘highrisk’ neuroblastoma, often shows itself at a late stage when it has spread around the body.
Perhaps your child has seemed ‘off-colour’ for a little while: initial symptoms are common ones – for example, aches and pains, loss of appetite or sweating.
However, the most common symptom when the cancer has developed is a lump or swelling in the child’s abdomen, as this is where the cancer usually starts. It can cause constipation and general discomfort and pain.
The site of the tumour may cause different symptoms. 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.
Other possible symptoms are:
a lump in the child’s neck, which can make them breathless or cause difficulty swallowing
bone pain and difficulty walking, if the cancer has affected their bones
numbness, weakness or loss of movement in the child’s lower body, if the cancer has affected their spinal cord
anaemia, bruising, bleeding and infections, if the cancer has affected their bone marrow
bluish lumps in their skin, if the cancer has spread to the skin
How is Neuroblastoma diagnosed?
Your child will undergo many different tests in order to confirm whether your child has cancer, what type and which treatment will be best.
The diagnosis of neuroblastoma is usually confirmed by tests at the specialist treatment centre. These centres are located in a network of 20 hospitals in the UK and Ireland with expertise in managing and treating all childhood cancers, including neuroblastoma.
Tests and assessments
Your child’s medical team will run a whole range of tests, assessments and scans to get an accurate diagnosis, including:
a clinical examination (the doctor’s physical examination)
blood and urine tests
special scans
X-rays
bone marrow tests
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. More information on each test can be found in our booklet, ‘A Parent’s Guide to Neuroblastoma’.
When will we get the results?
It can take several days for any tests to be completed and the results analysed. Undergoing these tests and waiting for results is an extremely anxious and stressful time for families. However, exact assessment of the extent of your child’s tumour before beginning treatment is very important. The results found will allow your child’s doctor to select the right type and length of treatment for your individual child.
What are the stages of neuroblastoma?
When your child is diagnosed, you will also be told what stage the cancer is at. This considers the size of the tumour and whether it has spread beyond the part of the body where it started.
Doctors recognise several categories of neuroblastoma that are grouped into different ‘stages’ and ‘risk groups’. The treatment your child has for their neuroblastoma depends on their tumour ‘stage’ and ‘risk group’. Knowing the stage of your child’s cancer helps doctors make sure your child is in the correct risk group so that they can give the right treatment and care.
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.
Staging system and classification
The International Neuroblastoma Risk Group (INRG) Staging System is used to assess the tumours of individual children to match the right treatment with the biology of their tumour. It takes into account risk factors and possible side effects.
INRG stage L1 (INSS stage 1) This means that the tumour is in one area (L = localised) and has not spread anywhere else in the body. This type of tumour is usually curable by surgery alone
INRG stage L1 (INSS stage 2) As with stage 1, the tumour is restricted to one area and has not spread anywhere else in the body. However, it may be larger than a stage 1 neuroblastoma and it may be more difficult to remove completely during an operation. Sometimes, a lymph node or some glands nearby may also be affected by the tumour. Stage 2 neuroblastoma is usually treated by surgery alone but depending on the site and results of certain genetic tests such as MYCN amplification, additional treatment like chemotherapy may be needed.
INRG stage L2 (INSS stage 3) This stage of tumour is also confined to the primary location and has not spread to other parts of the body. The tumour may be large and said to have crossed the ‘mid-line’ of the body. This means that the tumour has grown right across the child’s stomach or chest from the original side where it began to the other. This type of tumour would usually be difficult to safely remove with surgery alone. In other cases, the tumour may be wrapped around blood vessels that make it unable to be removed even though it is localised. Chemotherapy may initially be recommended to try to shrink the tumour before an operation, depending on the child’s age and the biology of the tumour. After the operation, radiotherapy and an oral medication called 13-cis-retinoic acid may also be recommended in some cases
INRG stage M (INSS stage 4) This means that the primary tumour may be any size, but some neuroblastoma cells have broken away and spread to other parts of the body, most commonly to the bones, bone marrow or liver. Depending on age, children with stage 4 neuroblastoma will likely require more intensive treatment. This will include chemotherapy to kill the tumour cells that have spread to different body parts, and to shrink the primary tumour for possible later removal by an operation.
INRG stage MS (INSS stage 4s) This is a type of neuroblastoma most often found in very young babies, under one year old. The tumour cells may have spread beyond the primary location to other parts of the body, but the cells usually behave in a less aggressive way than in an older child. The areas of the body affected by stage MS neuroblastoma, in addition to a small primary tumour (which is often in one of the adrenal glands), are most typically the liver (which on occasions can become extremely large), skin, distant lymph nodes and sometimes the bone marrow but not bone, lung or the brain.
Note: The older INSS classification of neuroblastoma stages is also included seen here in brackets. This is because some research study results that used INSS to determine a child’s risk group have not been published yet, and this information is needed to help decide on treatment options. For this reason, we have included them here as you might hear about both.
More information on each stage and risk group can be found in our booklet, ‘A Parent’s Guide to Neuroblastoma’.
What treatment is available for my child?
Once your child has a confirmed diagnosis of neuroblastoma, the medical team will be keen to get your child started on treatment as soon as possible.
Surgery
Your child’s tumour will be removed during an operation if it is possible and safe to do so. In some children, surgery may be involved at a later stage of treatment
Chemotherapy
This is the main treatment for intermediate- and high-risk neuroblastoma. Chemotherapy is the use of drugs to destroy cancer cells. They can be given in different ways but the most common way is intravenously – into a vein – whether through injections, cannula, or a line such as a central line, implantable port or PICC line. Chemotherapy is usually given as a combination of multiple different drugs.
High dose chemotherapy
After surgery, children with high-risk neuroblastoma undergo further treatment with ‘high-dose chemotherapy’, also sometimes called myeloablative therapy. As the treatment is intensive, your child may need to be in hospital for a period of about four to six weeks and be cared for in an isolation cubicle
Autologous stem cell transplant
Before high-dose chemotherapy is given, stem cells are collected, or ‘harvested’, from your child and frozen safely until they can be given back to your child after high-dose chemotherapy so that the bone marrow can recover.
Radiotherapy
Radiotherapy treats cancer by using high-energy rays to destroy cancer cells in a specific part of the body. Children with high-risk neuroblastoma will have radiotherapy after their tumour has been removed by surgery and after having high-dose chemotherapy. Some children with intermediate-risk neuroblastoma will also have radiotherapy after surgery but this decision is taken on an individual basis. Radiotherapy is painless and the machine does not touch your child – it is like having an ordinary x-ray.
Differentiation therapy/immunotherapy
For children with high-risk neuroblastoma whose tumours have responded to induction chemotherapy, surgery, high-dose chemotherapy and radiotherapy, additional treatment is recommended. This involves a drug called 13-cis-retinoic acid which ‘differentiates’ (turns cells from being cancerous to non-cancerous) any remaining tumour cells that may be present.
Alongside this, children will receive immunotherapy with a monoclonal antibody called anti-GD2 antibody. Immunotherapy treatment for neuroblastoma relies on your child’s own immune cells to kill cancer cells
You can be assured that if it is possible to treat your child with surgery alone or, very rarely, not to give any treatment at all, they will still be watched and examined very closely for many months.
No-one wishes to subject your child unnecessarily to the possible side effects of treatment. If it is considered that the risk of tumour cells spreading if very small, or that the tumour may go away on its own, minimal treatment will be advised.
It is most common, however, for a neuroblastoma to present as Stage 3 or 4 in the toddler or older age group, and this usually requires strong drug treatment from the outset.
What are the possible side effects of treatment?
Unfortunately, treatment can cause some side effects. Thankfully, these are mostly temporary and there are often ways of controlling or reducing them through supportive care. Your child may be well enough to play, have fun and a good quality of life during treatment. Your child’s cancer team will be able to tell you more about what side effects your child is most likely to have, and how they plan to manage them.
More information about possible side effects can be found in our booklet, ‘A Parent’s Guide to Neuroblastoma’.
Carolyn is supporting Neuroblastoma UK by taking on the Glasgow Kiltwalk 2024 in memory of her son, Matthew, who sadly passed away in November 2022 at the age of two and a half. She shares Matthew’s story here…