Outcome of Therapies for Supratentorial Ependymomas in Children

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.
Axial T1-weighted contrast MRI image showing recurrent anaplastic ependymoma, with multiple daughter nodules:

 

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).