Age
- Age at diagnosis is an important prognostic factor.
- The majority of children diagnosed at 12 months or younger will survive.
- Children less than one year with localized tumors have a five year survival greater than 90%, whereas in older children with advanced disease the long term survival is less than 30%, even with intensive therapy.
- Younger patients do tend to present with earlier stage disease.
Stage
Stage is an important predictor of outcome:
| Stage | 5 - 10 year Survival |
| 1 | Nearly all patients survive |
| 2 and 4S | 80% |
| 3 | 50 - 70% |
| 4 and over 1 year old | Only 5 - 10% |
MYCN Oncogene Amplification
- Genomic amplification of N-myc (or MYCN) is directly correlated with prognosis.
- If MYCN oncogene is amplified, patients tend to have advanced stage and rapid tumor progression.
- MYCN amplification is only rarely detected in localized neuroblastoma
Histopathology
- Shimada analyzed in CCG-3881 data and showed that unfavorable pathology was associated with advanced disease and less favorable outcome (except for Stage
I disease).
DNA Ploidy
- Flow cytometric techniques measure cellular DNA content or ploidy or cytogenetic
analysis of metaphase chromosomes. - A number of genetic abnormalities correlate with prognosis.
- Ploidy seems to override favorable clinical features.
- Hyperdiploid tumors do better than diploid tumors and respond better to chemotherapy. In infants with disseminated neuroblastoma, DNA ploidy accurately discriminated between good and poor responders to chemotherapy.
- MYCN gene amplification is more frequent in diploid than in hyperdiploid tumors.
Risk grouping for patients with neuroblastoma stratifies them into different prognostic categories.
| INSS Stage | Age | MYCN Status |
Shimada Histology |
DNA Ploid |
Risk Group |
| 1 | 0- 21 yrs | Any | Any | Any | low |
| 2A/2B | <1 y ≥ 1-21 y ≥ 1-21 y ≥ 1-21 y |
Any Non-Amp Amp Amp |
Any Any Fav Unfav |
Any - - - |
Low Low Low High |
| 3 | <1yr <1yr ≥ 1-21 y ≥ 1-21 y ≥ 1-21 y |
Non-Amp Amp Non-Amp Non-Amp Amp |
Any Any Fav Unfav Any |
Any Any - - - |
Intermediate High Intermediate High High |
| 4 | <1y <1y ≥ 1-21 y |
Non-Amp Amp Any |
Any Any Any |
Any Any - |
Intermediate High High |
| 4S | <1 y <1 y <1 y <1 y |
Non-Amp Non-amp Non-amp Amp |
Fav Any Unfav Any |
> 1 = 1 Any Any |
Low Intermediate Intermediate High |
Patients who present with elevated serum ferritin levels tend to have a poor prognosis.
