3 and 5-year progression-free survival rates are 58% and 38% respectively, and overall survival is 83% and 62% for the same time periods.
Absence of TP53 mutation may be associated with a more favorable outcome. One study showed a worse outome in tumors with loss of chromosome arm 12q 1).
The pediatric proclivity, in combination with a marked vascularity, renders an aggressive resection a difficult and often dangerous endeavor, with few treatment options.
Gross total resection is the most consistent predictor of survival 2).