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

Abstract

BACKGROUNDThe 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.METHODSThe 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.RESULTSThe 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.CONCLUSIONSThe 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. Cancer 2004. © 2004 American Cancer Society.

Supratentorial ependymomas: Prognostic factors and outcome analysis in a retrospective series of 46 adult patients

“…Age <55 years was associated with a better prognosis in multivariate analysis in terms of OS but not for PFS. These results are consistent with those reported by Reni et al but differ from those reported by Guyotat et al 1,3 These are the only 2 reports in the literature that analyze survival in adult intracranial ependymomas with regard to different age groups. According to the median age of our population 2 cutoff ages were chosen: age 45 years and age 55 years.…”

“…5,9,18 The prognostic value of histologic findings remains a controversial issue that most likely is attributable to sample size, variability in the definition of anaplasia, discrepancies in histologic diagnoses, and the inclusion in some series of ependymoblastomas and subependymomas, which exhibit different biologic behavior and should be analyzed separately. 1,4,5,9,[19][20][21][22] It is important to emphasize that, in the current study, 57 of 103 patients (55.3%) initially had an incorrect diagnosis of ependymoma. Four classes of pathologic misdiagnoses can be distinguished.…”

“…8 In a recent series, Reni et al did not observe a correlation between histologic grade and survival. 1 However, in their study, no central pathologic review was planned, and the role of histology in predicting outcome may have been masked by the various definitions of anaplasia used by the pathologists involved in the diagnostic process. The lack of impact of tumor grade on survival in their report also may be attributable to the relatively small number of anaplastic ependymomas.…”

Supratentorial hemispheric ependymomas: an analysis of 109 adults for survival and prognostic factors

“…32 Anaplastic histology was found in 62% of hemispheric ependymomas, a larger percentage than previously reported for intracranial ependymomas (23%-54%). 2,14,21,38,46,47,53 Several series have also found a higher percentage of WHO Grade III tumors in hemispheric ependyomas. 9,22,47,55 Guyotat and colleagues found that all 10 hemispheric ependymomas in their case series of 34 intracranial ependymomas had WHO Grade III pathology.…”

“…8,9,[19][20][21][22][46][47][48]54 Evidence supports the idea that supratentorial ependymomas have a worse prognosis than infratentorial tumors do in adults. 9,19,20,23,38,45,46 Additional data indicate that patients with hemispheric ependymomas have decreased PFS and OS compared with tumors occurring in the third or lateral ventricles. 9,45 Although often considered an intraventricular tumor, more than half of supratentorial epabbreviatioNs EOR = extent of resection; GTR = gross total resection; OS = overall survival; PFS = progression-free survival; STR = subtotal resection.…”