What is high-risk neuroblastoma?

Neuroblastoma is a rare cancer that develops in very early nerve cells called neuroblasts. These cells form while a baby is growing in the womb and are part of the sympathetic nervous system. Most neuroblasts mature and disappear before birth. If some of these immature cells grow or divide in an uncontrolled way, they can form a tumour. 

The name comes from: 

• Neuro – nerve 
• Blast – immature cell 
• Oma – tumour 

High-risk neuroblastoma refers to a subset of the disease that has specific features. According to the International Neuroblastoma Risk Group (INRG) system used by doctors, high-risk usually means the cancer has spread to other parts of the body (metastasised), the child is over 18 months old, or the tumour has certain genetic features that make it more aggressive. 

How doctors decide if neuroblastoma is high risk 

Doctors look at several factors together: 

• Where the tumour is and whether it has spread: High-risk neuroblastoma often means the cancer is not just in one place (INRG “M” stage) but has spread to distant parts of the body. 

• Age at diagnosis: Children over 18 months are more likely to be in the high-risk group. 

• Tumour biology: Certain genetic changes, like MYCN amplification, or other unfavourable features, make the tumour more aggressive. 

• Imaging features (IDRFs): These show if the tumour is close to or involving important structures within the body, which can affect risk and treatment. 

What high risk means for treatment 

High-risk neuroblastoma can be more difficult to treat due to its aggressive nature. Children usually need more intensive treatments. This may include: 

• Chemotherapy 

• Surgery to remove the tumour 

• High-dose chemotherapy with stem cell rescue (transplant) 

• Radiotherapy 

• Immunotherapy 

• Sometimes newer treatments or clinical trials 

Your child’s medical team will explain the treatment plan and what it means for your family. 

Read more about treatment for high-risk neuroblastoma