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Risk groups and staging for neuroblastoma

Doctors group children with neuroblastoma into risk groups, according to their risk of the cancer coming back after treatment. The risk groups are called:

  • low risk
  • intermediate risk
  • high risk

The treatment your child has for their neuroblastoma depends on which risk group they are in. Doctors use the cancer stage as well as the child’s age and results of other tests to make a decision about treatment.

Staging neuroblastoma

The stage of a cancer tells you the size and whether it has spread. Knowing the stage of the neuroblastoma helps doctors make sure your child is in the correct risk group.

There are a number of different staging systems. The newest system uses imaging tests such as CT scans, to look for particular risk factors. These factors are a sign that the neuroblastoma is more difficult to treat. For example, doctors look at whether the tumour is wrapped around an organ or large blood vessels. These risk factors are called image defined risk factors.

The older system is shown first here and the newer staging system is in brackets.

Stage 1 and 2 (L1)

The tumour is in one area of the body and hasn’t spread anywhere.

Stage 3 (L2)

The tumour has spread into nearby structures but not far. Tests show at least one image defined risk factor.

Stage 4 (M)

The cancer has spread to parts of the body that are some distance from where it started.

Stage 4s (MS)

This a special case, as these children have a better outlook than other stage 4 patients. Stage 4s (MS) disease occurs in very young babies or infants. It doesn’t occur in children over 18 months old.

The neuroblastoma might have spread to the liver or skin, but not to the bones. It also means less than 10% of the cells in the bone marrow are neuroblastoma cells.

Neuroblastoma risk groups

Doctors take into account a number of different things when deciding on a risk group. It’s likely that as our understanding of neuroblastoma develops, doctors will add or take factors out, to keep the risk groups up to date.

Age

Children diagnosed with neuroblastoma when they are younger than 18 months are at lower risk.

What the tumour looks like under a microscope (histology)

Tumours with more normal looking cells tend to be lower risk.

Genetic changes

A gene called MYCN controls cell growth. Some neuroblastoma tumours have too many of these genes. This means that the cells grow quickly and are less likely to mature. Tumours with too many MYCN genes tend to be higher risk.

Another gene change doctors look for, is how much DNA there is in the cells. Genes are made of DNA and they are grouped together to make chromosomes. Changes in chromosomes and how much DNA there is in a cell can affect how well treatment works.

Treatment by risk group

We have information on what treatment to expect depending on what risk group your child is in.

Remember this is only a guide, your doctor and specialist nurse will give you a clear individual treatment plan for your child. Ask them as many questions as you need about staging, risk groups and the science of neuroblastoma. It’s very complex and no one would expect you to understand it all straightaway.

Last reviewed: 
16 May 2018
  • Overview and recent advances in the treatment of neuroblastoma

    SB Whittle and others

    Expert Review of Anticancer Therapy, 2017

    Volume 17, Number 4

  • Cancer in children – clinical management (6th edition)

    MCG Stevens, HN Caron and A Biondi

    Oxford University Press, 2012

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