Patients are stratified into different prognostic categories or risk groups.
Therapy design is based on the patient’s risk status.
Risk categories depend on:
- Pathology
- histology
- N-MYC status
- DNA ploidy
- Diagnostic imaging results
Neuroblastoma Risk Categories:
INSS Stage |
Age |
MYCN |
Shimada |
DNA |
Risk Group |
1 |
0- 21 yrs |
Any |
Any |
Any |
low |
2A/2B |
<1 y |
Any |
Any |
Any |
Low |
3 |
<1yr |
Non-Amp |
Any |
Any |
Intermediate |
4 |
<1y |
Non-Amp |
Any |
Any |
Intermediate |
4S |
<1 y |
Non-Amp |
Fav |
> 1 |
Low |
The risk group classification determines the treatment for each individual patient.
More than half the children diagnosed with neuroblastoma will have high risk disease with a poor prognosis.
Recent studies suggest that toddlers aged 12-18 months with:
- Stage 4 disease with N-MYC non-amplified, hyperdiploid, favorable histology have an event free survival of 90%
- Stage 3 disease with N-MYC non-amplified, unfavorable histology have an event free survival of 100%
For this reason, this cohort of patients will now be treated according to the intermediate risk protocol.
Age and stage are major prognostic factors in neuroblastoma.
Simplified overview of Neuroblastoma Risk Categories:
| Risk Category | Definition |
Low risk |
Age less than 1 year ( now extended to 420 days)
Stage 1 (with local resection), Stage 2A partially resected, Stage 4.
N-myc - non amplified
Hyperdiploid, near triploid DNA ploid
High TRKA expression
Lack the 1p loss of heterozygosity or 14q heterozygosity
|
Intermediate Risk |
Age over 1 year
Stage 2B, Stage 3, Stage 4
N-myc non amplified
Near diploid and near tetraploid DNA ploid
TRKA variable
1p and 14q loss of heterozygosity variably present
|
High Risk |
Age over 1 year
Stage 3 and Stage 4
N-myc ampified
Near diploid and tetraploid DNA ploid
TRKA expression low
1p and 14q loss of heterozygosity variably present
|
