The Survival and Prognostic Factors of Supratentorial Cortical Ependymomas: A Retrospective Cohort Study and Literature-Based Analysis

Abstract

Aim: Survival rates and prognostic factors of cortical ependymomas (CEs) remain elusive. This study aimed to perform a comprehensive analysis of prognostic factors, treatment, and outcomes for patients with CEs based on institutional and literature case series. Materials and Methods: Thirty patients with CEs from our department were included in this study. Furthermore, a systemic review of the literature yielded an additional 106 patients with CEs. Clinical data including patient age, sex, symptoms, tumor location, World Health Organization (WHO) grade, extent of surgery, radiation, recurrence, and survival were recorded and statistically analyzed. Results: From January 2009 to October 2019, 30 (4.2%) cases were diagnosed as CEs in our department. These series consisted of 19 males and 11 females, 10 continuous patients after 2017 screened for C11orf95-RELA fusion, and 9 patients (90%) were RELA fusion positive. During the follow-up period, nine (30%) patients depicted tumor recurrence or progression; four (13.3%) patients died of tumor progression. The literature review yielded 106 CE cases, with additional 30 cases of our own collected for further analysis. Of these 136 cases, the frontal lobe (40%) was the most common location, and the average age was 22.6 ± 17.6 years. Anaplastic histology/WHO grade III tumors were identified in 68 (50%) patients. Statistically analysis demonstrated that extent of surgery and WHO tumor grade were significant prognostic factors in Kaplan-Meier logrank testing and Cox proportional hazards models. Gross total resection (GTR) predicted longer progression-free survival (PFS) [P = 0.013, hazard ratio (HR) = 3.012, 95% confidence interval (CI) = 1.257-7.213] and overall survival (OS)

Pure Cortical Ependymoma With RELA-Fusion Positive in a Young Adult

“…In 2019, Matsumoto et al [ 15 ] reported their 8 cases and 84 cases previously reported CE cases. Their results were similar to those of Wang et al [ 14 ]. They performed Sanger sequencing of cDNA for finding C11orf95-RELA -fusion in 5 of their 8 cases that the test was available, and all 5 cases were fusion positive.…”

“…In several literatures, CEs are defined as EPNs that are located in the cerebral cortex and have no connection to the ventricular lining and that account for only about 3.5% to 5.7% of all EPNs [ 2 3 14 15 ]. Especially, in some literatures like this case, it is called pure CEs to emphasize that they are limited only to the cerebral cortex [ 5 16 ].…”

“…Currently, CEs have been reported about 100 cases. In 2020, Wang et al [ 14 ] reported a cohort study on 106 cases that have been reported till that date and 30 cases of CEs of their institute. In their study, seizure is the most common symptom, the frontal lobe or involving the frontal lobe are the most common site of CEs and relatively higher rate of C11orf95-RELA -fusion than that of other STEs that fusion rate is known as about 70%.…”

“…In their study, seizure is the most common symptom, the frontal lobe or involving the frontal lobe are the most common site of CEs and relatively higher rate of C11orf95-RELA -fusion than that of other STEs that fusion rate is known as about 70%. They performed C11orf95-RELA -fusion test in 10 of their 30 cases that the test was available, and 9 cases were confirmed to be fusion positive by FISH [ 14 ]. The prognosis of CEs was favorable, whereas a poorer prognosis was expected than that of other STEs because CEs have higher rate of C11orf95-RELA -fusion and they have anaplastic features [ 14 ].…”

“…They performed C11orf95-RELA -fusion test in 10 of their 30 cases that the test was available, and 9 cases were confirmed to be fusion positive by FISH [ 14 ]. The prognosis of CEs was favorable, whereas a poorer prognosis was expected than that of other STEs because CEs have higher rate of C11orf95-RELA -fusion and they have anaplastic features [ 14 ].…”