Outcome After Surgery
Prognostic factors
- Histological grade: Anaplastic tumors (WHO grade III) are more likely to occur in a supratentorial location. One study showed a 50% recurrence rate for anaplastic ependymomas despite gross total resection and radiation (19).
- Age: Children younger than 3 years have poorer 5-year survival and progression-free survival rates compared to those who are older than 3 years (24). This may be due to incomplete surgical resections or a delay in the administration of radiation.
- Extent of surgical resection: A complete resection infers a 5-year survival rate of 60–80% for ependymomas in general and a 70–80% disease-free survival rate at 48–120 months for patients with totally resected supratentorial ependymomas (4, 16). Only 42–62% of patients experience a complete resection. The 5-year survival rate is only 20–22% for incompletely resected ependymomas regardless of their locations.
Outcome After Nonsurgical Treatments
Radiotherapy
- No role for radiotherapy alone: The current strategy is to irradiate after surgery (see above). Radiotherapy should not be unduly delayed, preferably not later than 1 year after diagnosis.
Chemotherapy
- No role for chemotherapy alone: Carboplatin and etoposide are frequently used because of CNS penetration. The current strategy is to use chemotherapy to make second-stage resection more amenable to complete resection (18, 25).
- Possible role as preoperative treatment: In several series a complete surgical resection of the tumor was achieved in 75% of patients who were pretreated with chemotherapy (17, 18).
Outcome After Multimodal Therapies
Surgery plus postoperative radiotherapy
- 5-year survival rates of 40–88%: The overall survival rate for ependymomas, regardless of their location, is 40–88% with combined surgery and radiation as opposed to 17–38% for those patients managed with surgery alone (1, 21).
- Neurological function: Common sequelae include abnormal gait, difficulties with fine motor function, and cognitive impairment (from radiotherapy), occurring in 35–55% of cases. 30% of patients require placement of a ventriculoperitoneal shunt (22).