A multicenter study of the prognosis and treatment of adult brain ependymal tumors

Abstract

Background: The current analysis of outcomes in a large series of adult patients with intracranial ependymal tumors contributes to the characterization of the primary prognostic factors and to the therapeutic management of this rare disease, for which limited information is available in the literature.

Methods: The authors analyzed data on patient and tumor characteristics, treatment, and survival in a series of 70 patients age > 17 years with pathologic diagnoses of brain ependymal tumors from 4 institutions.

Results: The 5- and 10-year overall survival (OS) rates (+/- standard errors) were 67% +/- 6% and 50% +/- 8%, respectively. The 5- and 10-year failure-free survival (FFS) rates were 43% +/- 7% and 24% +/- 6%, respectively. Younger age and infratentorial tumor location were associated with longer survival. Among patients with Grade 2 ependymoma (n = 51), 21 (41%) received no postsurgical treatment. These 21 patients had a 5-year OS rate of 78% +/- 10% and a 10-year OS rate of 68% +/- 13%; the 5- and 10-year FFS rates for these patients were 47% +/- 12% and 12% +/- 11%, respectively. Twenty-six patients with Grade 2 ependymoma (51%) received postoperative radiotherapy (RT). These 26 patients had a 5-year OS rate of 71% +/- 9% and a 10-year OS rate of 59% +/- 11%; the 5- and 10-year FFS rates for these patients were 54% +/- 10% and 34% +/- 10%, respectively. Among patients with Grade 2 ependymoma, neither OS nor FFS differed significantly between those who did not receive postoperative RT and those who did; however, these two groups were heterogeneous with respect to prognostic factors. On multivariate analysis, RT use exhibited a trend toward improved OS and was significantly predictive of improved FFS.

Conclusions: The current analysis does not rule out the possibility that deferral of RT at the time of recurrence could have a detrimental effect on FFS or OS in patients with Grade 2 ependymoma, regardless of the degree of ablation. The role of postoperative RT for patients who undergo imaging-based macroscopic total resection remains to be addressed.