Doctors will “stage” the disease once they have reviewed all the scans and results. This means an exact assessment is made as to how far the tumour has spread in the body. The different stages of neuroblastoma have different treatments, as they carry with them different risks. There are, however, other important factors which also affect the prognosis and these include:
- the age of the child (children diagnosed under 18 months generally have less aggressive disease)
- the appearance of the tumour cells under the microscope (histopathology)
- the genetic makeup of the tumour cells – in particular a gene called “N-Myc” or “MycN”
After all the results, including the tumour biopsy, have been received the oncologist will classify the child’s neuroblastoma as low, intermediate or high risk and will provide details of the treatments required (often referred to as the ‘protocol’). It is important to note that if the tumour biopsy shows very aggressive disease (eg the gene called N-Myc is “amplified”) then staging becomes less relevant.
Amplified N-Myc means the tumour is very aggressive and high risk protocol will automatically be followed even if the tumour has not spread to other parts of the body.
For many years the International Neuroblastoma Staging System (INSS) has been used to assess individual children and these descriptions (stage 1, 2, 3, 4 and 4S) are likely to continue to run alongside a newer International Neuroblastoma Risk Group Staging System (INRSS). The new system is aimed at getting a better match between the risks and the side-effects of treatment with the risk of the disease. The approximate INRSS stage is shown in brackets against the INSS stage below:
- Stage 1 Neuroblastoma (INRSS stage L1)
- Stage 2 Neuroblastoma (INRSS stage L1)
- Stage 3 Neuroblastoma (INRSS stage L2)
- Stage 4 Neuroblastoma (INRSS stage M)
- Stage 4S (INRSS stage MS)
The tumour is fairly small and confined to one site and it has not spread anywhere else in the body and it is able to be removed completely by an operation. This type of tumour is usually curable by surgery alone.
As with stage 1, the tumour is confined to one site and has not spread to distant parts of the body. However it may be larger than a Stage 1 neuroblastoma and at operation, it may be more difficult to remove completely. Sometimes a lymph node or some glands near to the stage 2 neuroblastoma may also have been affected by the tumour. Stage 2 neuroblastoma may be treated with surgery alone or with chemotherapy.
The tumour is also confined to the primary site and has not appeared in other distant parts of the body. The tumour may be very large and said to have crossed the ‘mid-line’ of the body. This means that the tumour has grown right across the child’s abdomen or chest from the original site where it began. In the case of stage 3 tumours, chemotherapy treatment will often be initially recommended to try and shrink the tumour for subsequent surgical removal. Further treatment will depend on the other tests described above.
This means that the primary tumour may be of any size but some neuroblastoma cells have broken away and spread to other organs of the body, most commonly bones, bone marrow or liver. Drug therapy (chemotherapy) will usually be recommended for a stage 4 neuroblastoma. This has 2 aims:
a) to kill tumour cells that have spread to different body organs
b) to shrink the primary tumour for later removal by an operation
After surgery, further drug therapy is given. Other treatment may then follow depending on the results of the tumour biopsy and how aggressive the neuroblastoma is.
This is a special type of tumour found in very young babies, under one year old. The tumour cells may have spread beyond the “primary site” to other parts of the body but the cells usually behave in a less aggressive fashion than in older children. The tumour may even shrink and disappear without treatment. Alternatively, low doses of either radiotherapy or chemotherapy may be used.
Ganglioneuroblastoma is another type of tumour, but a close relative of neuroblastoma. It can present in any age group but the cells of this tumour are more ‘mature’ (less likely to spread) than those of ordinary neuroblastoma.
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