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Prognosis

Much of the literature on ependymoma prognosis is confounded, as adult and pediatric cases are often combined. If one were to look at the outcome of pediatric cases alone, the rate of survival would be much lower. The five-year overall survival rate for pediatric ependymoma is 50-64% and that for progression-free survival is 23-45%.

Several factors are associated with the prognosis of pediatric patients. Surgical resection is considered the most important of these. Many new studies indicate gross total resection does improve both overall and progression-free survival.

There is also an increased risk of recurrence when there is brainstem invasion, as indicated by neuroimaging or neurological signs. This may also suggest cranial nerve involvement.

Collins' rule appears to be valid in labeling many cases as cured, but there are exceptions and late recurrence has been known to occur.

Table 8. Prognostic factors and their predictions for the outcome of the disease.
Prognostic Factor Association with Outcome
Surgical resection An improved survival rate is associated with gross total resection.
Age Younger patients have a worse prognosis.
Children under age 5 at diagnosis are particularly at risk.
Tumour grade Worse outcome with higher tumour grade.
Tumour location Infratentorial tumours have greater risk than supratentorial tumours.
Subarachnoid seeding High-grade tumours are more often associated with seeding
Radiation dose There is an increased rate of occurrence with lower radiation dose:
  • 51% survival with 4500cGy
  • 18% survival with <4500cGy