Ependymoma is a primarily pediatric cancer and the role of chemotherapy is not well defined. In 1998, an extensive review and analysis of all published literature on the topic of intracranial ependymoma highlighted the difficulty associated with extrapolating data from single-institution studies.
Forty-five series were reviewed, including more than 1400 children. The largest series reported on 92 patients, and the accrual rate ranged from 0.32-12 patients per year. Notably, the extent of surgical resection was the only reported prognostic factor in these series that was consistently found to be a valid predictor of outcome.
These findings were confirmed by a prospectively randomized trial published that same year evaluating Children’s Cancer Group Protocol 921. Predictors of long-term survival included an estimate of the extent of resection made at surgery (total compared with less than total, P=0.0001) and the amount of residual tumor on postoperative imaging as verified by centralized radiologic review. Other factors, including centrally reviewed tumor histopathologic type, location, metastasis, and tumor (M and T) stages, patient age, race, gender, and chemotherapy treatment regimen were not found to be correlated significantly with long-term survival.Currently, no role exists for adjuvant therapy of spinal ependymoma after complete surgical resection. For patients who have postoperative residual tumor or early recurrence, radiation is considered on the basis of the individual patient’s medical condition and neurological status.
In a 2001 abstract, the authors conclude, “a significant proportion of children with ependymoma can avoid radiotherapy with prolonged adjuvant chemotherapy.” The conclusion of this abstract and article gives an impression that chemotherapy can be used so that irradiation can be avoided. In their study, the authors documented that 40% and 23% of children were spared from radiotherapy at 2 and 4 years, respectively, from the initiation of chemotherapy. This has engendered a lively debate that has not ended. NCCN does nto recommed adjuvant chemotherapy and for recurrence or anaplastic disease recommends, CPTII, temozolamide, notrosoureas, PCV or paltinum based regimens for 2nd or 3rd line therapy.
NCCN recommends tmozolamide, etoposide, nitrosoureas, platins, singly or in combination for recurrent or metastatic disease.
R.M. Green, MD Bevacizumab for recurrent ependymoma
Neurology – Volume 73, Issue 20 (November 2009)
nccn.org, Adult Ependymoma