Adult intracranial ependymomas (EPNs) are extremely rare brain tumors. Currently, clinical and molecular factors that could inform individualized treatment strategies are still lacking for EPNs in this age group. The aim of this study was to investigate potential prognostic indicators and rational therapeutic management in a large cohort of adult intracranial EPNs. Adult patients who underwent resection of World Health Organization (WHO) grade II or III intracranial EPNs were included. The demographic features, clinicopathologic manifestations, molecular subgroups, and outcomes were retrospectively analyzed. Overall survival and progression-free survival were calculated using the Kaplan-Meier analysis. Potential prognostic indicators were identified using multivariable Cox proportional hazards model. This cohort included 236 adult patients with a mean age of 36.2 years (range: 18 to 72 y) at diagnosis. The tumor location was supratentorial (ST) in 102 (43.2%) and infratentorial in 134 (56.8%). Pathologic analysis revealed 43.1% of ST-EPNs with RELA fusion and 88.1% of posterior fossa ependymomas (PF-EPNs) with positive H3K27me3 staining. Gross total removal was achieved in 169 cases (71.6%). During follow-up, 97 (41.1%) patients had disease progression and 39 (16.5%) died. Kaplan-Meier analysis showed that patients with H3 K27me3-positive PF-EPN had excellent survival, whereas patients with RELA fusion-positive ST-EPN or H3K27me3-negative PF-EPN had poor prognosis (progression-free survival: P=1.3E−16, overall survival: P=2.5E−12). Multivariate analysis showed that molecular subgroup, extent of resection, and Ki-67 index were strong independent prognostic indicators. In conclusion, our study provides essential information on the prognostic prediction of adult intracranial EPNs that will assist in establishing appropriate risk stratification and individualized treatment strategies in future clinical trials. F.Z. and T.W. contributed equally. F.Z. and P.-n.L.: contributed in conception and design. T.W., B.W., and L.L.: contributed in provision of study materials and/or patients. S.-W.L., J.Z., and S.Z.: collected and assembled the data. T.W., F.Z., and L.-m.W.: carried out data analysis and interpretation. F.Z. and T.W.: wrote the manuscript. Conflicts of Interest and Source of Funding: Supported by the Platform Construction of Basic Research and Clinical Translation of Nervous System Injury (2020) (number: PXM2020_026280_000002, Beijing Municipal Health Commission to P.L.), and Beijing Program Foundation for the Talents (grant number: 2016000021469G216 to F.Z.). The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article. Correspondence: Pi-nan Liu, MD, PhD, Laboratory of Neural Reconstruction, Beijing Key Laboratory of Central Nervous System Injury, Beijing Neurosurgical Institute, Capital Medical University. No. 119, South 4th Ring Road, Fengtai District, Beijing 100070, China (e-mail: pinanliu@ccmu.edu.cn). Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
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