The natural history of supratentorial PNETs is difficult to predict with certainty because:
- Supratentorial PNETs are rare
- Controversy regarding histological and pathological classification
Long-term survival in supratentorial PNET patients is poor14 and significantly worse than medulloblastoma.
Survival is also influenced by different prognostic factors, (both tumor related and non-tumor related). These factors play an important role in treatment planning25.
Summary of prognostic factors:
Factor |
Favourable |
Unfavourable |
Age
|
Aged 3 and older
2 year survival rate= 72%
5 year survival rate= 50% |
Under age 3
2 year survival rate= 28%
5 year survival rate= 5.8%
*Most likely due to limited use of RT for younger children11 |
Location |
Pineal region |
Non-pineal Deep region of CNS
|
| Extent of resection | Gross total resection or near total resection |
Subtotal, partial or biopsy type resection |
| Spread | Localized disease | Evidence of dissemination17 |
Treatment method |
Aggressive multimodality treatment |
The use of multimodality treatment is limited (eg. treatment complications such as infection) |
| Dose and volume of RT | Treatment to whole CSA Dose of 3500 cGy to CSA |
No RT or low dose RT to CSA |
Histology |
Not shown to be a significant prognostic marker10 |
|
