Once it has been confirmed that your child has neuroblastoma and the disease has been staged there is a distinct treatment pathway that aims to first reduce the size of the primary tumour, remove as much of the tumour as possible with surgery, achieve remission and then ensure the cancer does not return. The treatment is different for each stage of the cancer, as shown in the Diagnosis section. Here we will focus on the journey ahead for those diagnosed as “High Risk”, such as those with stage 4 neuroblastoma.
The main aim of induction therapy is to reduce the size of the primary tumour to make it easier to remove as much of it as possible during surgery. There are different chemotherapy regimens used in the induction phase but the most common in the UK are listed below:
- This is a combination of five agents (cisplatin, vincristine, carboplatin, etoposide and cyclophosphamide) that is delivered directly into the child’s bloodstream through their central line
- This therapy is administered in eight cycles, separated by intervals of 10 days and is completed within 70 days of the first treatment
- In high-risk patients the use of Rapid COJEC is to give high doses of chemotherapy over a shorter time which may improve survival
- Modified N7 uses the drugs cyclophosphamide, doxorubicin, vincristine, cisplatin and etoposide
- The treatment lasts 3-4 days every 3 weeks, this is one cycle
- There are five cycles of this chemotherapy
- Your child will also be given a “growth factor” known as G-CSF as an injection
- These injections are given daily between cycles to reduce the risk of infection
During induction chemotherapy your child will have some of their “peripheral haematopoietic stem cells” taken from their blood. These are cells that can develop into any of the different types of blood cells and so are very important for carrying oxygen, fighting infection, and preventing bleeding. During chemotherapy these cells can get damaged and this can limit the amount of therapy the child can have as it takes some time for the blood to recover. Taking some cells while they are healthy, and storing them during the induction phase means that these cells can be replaced later, therefore improving the child’s ability to make new blood cells.
After induction chemotherapy surgery will be performed to remove as much of the primary tumour as possible. At this point the hope is that the tumour will have reduced in size since diagnosis, so that the surgery will be as successful as it can be. The surgeon will remove the tumour along with a margin of healthy cells while trying to cause little disruption to surrounding structures and organs.
The role of consolidation therapy is to ‘mop-up’ any remaining cells that might be left over following previous chemotherapy and surgery. This chemotherapy is known as myeloablative and is high-dose. The term myeloablative refers to the depletion of the blood-producing cells in the bone marrow as a side-effect. The drugs used in this process are called busulfan and melphalan.
After this chemotherapy is complete, the stem cells that were taken from the child during induction therapy will be replaced in a process known as stem cell rescue. This process aims to restore the blood-producing cells in the bone marrow using the child’s own cells, meaning that there is no risk of rejection as there could be with donor stem cells.
Once these processes are complete, dependant on age, your child may undergo radiotherapy on the area around their primary tumour site. Again, this is to try to ensure any cancer cells left over from surgery are killed off.
The aim of maintenance therapy is to keep your child in remission and keep the cancer away. There are a couple of aspects to this part of treatment that have slightly different roles:
- Aims to revert cancer cells to “normal function”
- Use of oral drug isotretinoin (13-cis-retinoic acid)
- Aims to help the body’s own defences turn against the neuroblastoma cells using antibodies
- This should keep the cancer from returning as the body will learn to recognize and kill the cells
After maintenance therapy many families look towards clinical trials to keep the cancer at bay for as long as possible.
 Peinmenn et al. (2013), Rapid COJEC versus standard induction therapies for high-risk neuroblastoma (protocol), http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD010774/pdf, accessed 07/12/2015
 NIHR, (2015), APN311 for high risk neuroblastoma in children and adolescents – first line, file://vsbs2011/RedirectedFolders/jcalvert/Downloads/APN311-May-2015.pdf, accessed 07/12/2015