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  1. About Neuroblastoma
  2. About Neuroblastoma Home

Risk Groups and Staging

Neuroblastoma varies greatly from case to case, and this means that the prognosis is unique to a child’s particular circumstances. Similarly, the treatment pathway for neuroblastoma is not the same for every child, and it is common to find that they will not follow what is considered the ‘typical’ journey. 

Stages of neuroblastoma 

There are different 'stages' of neuroblastoma which describe how far it has grown and spread, and clinicians use this to decide the best treatment paths for the child. 

Stage 1: The cancer is localised and has not spread. It can be completely or almost completely removed by surgery. 

Stage 2: The cancer is localised and has not spread, but it cannot be completely removed by surgery. This is often due to the position of the tumour meaning total tumour removal would cause damage to organs or major blood vessels. 

Stage 3: The tumour has begun to spread to nearby lymph nodes but not distant parts of the body. 

Stage 4: This is when the tumour has spread to distant parts of the body. This stage is usually designated as 'high-risk' and will usually be treated with chemotherapy, surgery, radiation, stem-cell transplant and immunotherapy. Around half of children have disease that has already spread by the time of diagnosis, so whilst it may be alarming to be diagnosed at Stage 4, this is not uncommon due to the nature of neuroblastoma which makes it difficult to spot. 

Stage 4s: This is a stage unique to this type of childhood cancer and doesn't occur in children over 18 months old. It refers to a type of the disease that presents as a primary tumour and may have spread to local lymph nodes, but only on one side of the body. There will be no spread to the bones and less than 10% of bone marrow cells will be neuroblastoma. This type of disease has been known to spontaneously regress into a benign state and so often the only course of action will be to maintain close observation of the child to ensure nothing changes. 

Risk groups 

As well as defining the stage of the cancer, your child will also be given a risk group which mostly relates to their risk of the cancer coming back and how far it has spread. It also determines the treatment they will receive. 

There are three risk groups: 

Low-risk neuroblastoma: This normally applies to Stage 1 patients, and most Stage 2 patients who are less than 12 months old and/or have no unfavourable genetic mutations. 

Intermediate-risk neuroblastoma: This usually includes Stage 3 and 4 patients, who are less than 12 months old and have no unfavourable genetic mutations. 

High-risk neuroblastoma: This usually includes Stages 2, 3, and 4 where the patient is older than 12 months and/ or has unfavourable genetic mutations or tumour histology. 

There is a vast difference between treatments and outcomes for low, intermediate and high-risk neuroblastoma. The treatment for low-risk disease is much less intensive- in some cases the tumour can go away on its own, not needing treatment but just close monitoring. More extensive treatment may be needed for intermediate disease, but this will depend on your child’s particular circumstances. 

Unfortunately, high-risk neuroblastoma requires particularly intensive therapies which can make your child quite ill, and in some cases leads to life-long side effects. Even within this risk group, there can be much variation, and so the treatment pathway your child takes may look different to the ‘typical’ pathway, or others that you are aware of. Your treatment team will always be following the best options for your child’s circumstances. 

Published: 22nd March, 2022

Author: Emily Hall

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Solving Kids’ Cancer UK is a registered charity in England and Wales (1135601), and in Scotland (SCO45094). It is a company limited by guarantee in England and Wales (7208648). The charity and company is currently operating as Solving Kids’ Cancer.