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RELA Fusion in Supratentorial Extraventricular Ependymomas: A Morphologic, Immunohistochemical, and Molecular Study of 43 Cases.

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机构: [1]Departments of Pathology, Xuanwu Hospital Capital Medical University, Beijing [2]Departments of Radiology, Xuanwu Hospital Capital Medical University, Beijing [3]Departments of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing [4]Department of Pathology, Beijing Fengtai Hospital, Beijing [5]Department of Pathology, Peking University International Hospital, Beijing [6]Department of Pathology, Guangdong Sanjiu Brain Hospital, Guangzhou [7]Department of Pathology, Guangdong Provincial Peoples Hospital, Guangzhou [8]Department of Pathology, Jining People’s Hospital, Jining, China
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Supratentorial extraventricular ependymomas (STEEs) are relatively rare ependymomas, and their pathologic and genetic characteristics are still poorly understood. The aim of this study was to determine the histologic, immunohistochemical, and RELA fusion features, as well as to clarify in more detail the clinical courses of STEEs. Data from a total of 43 patients with STEEs was analyzed retrospectively. The status of RELA fusion was evaluated using fluorescence in situ hybridization. The expression levels of L1CAM, p65, cyclin D1, and p53 were assessed using immunohistochemistry. Progression-free survival and overall survival were calculated via Kaplan-Meier estimation using the log-rank test. Among all 43 STEEs, 65.1% (28/43) are positive for RELA fusion. Interestingly, almost half of the patients with RELA fusion-positive ependymomas are adults (13/28), and 89.3% (25/28) cases are anaplastic ependymomas, which suggests that RELA fusion testing is necessary in adults with STEEs. We investigated the immunohistochemical status of p65, L1CAM and CCND1 protein expression for their ability to predict RELA fusion status. RELA fusion-positive STEEs are frequently associated with expression of p65 (85.2%), L1CAM (85.2%), and CCND1 (81.5%). The accuracy of predicting RELA fusion status was much higher when the expression of p65 and L1CAM was combined, that is, when both were immunopositive. The status of RELA fusion, p53 overexpression, and extent of tumor resection are significantly associated with prognosis.

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出版当年[2018]版:
大类 | 2 区 医学
小类 | 1 区 外科 2 区 病理学
最新[2023]版:
大类 | 1 区 医学
小类 | 1 区 病理学 1 区 外科
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出版当年[2017]版:
Q1 SURGERY Q1 PATHOLOGY
最新[2023]版:
Q1 SURGERY Q1 PATHOLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2017版] 出版当年五年平均 出版前一年[2016版] 出版后一年[2018版]

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第一作者机构: [1]Departments of Pathology, Xuanwu Hospital Capital Medical University, Beijing
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通讯机构: [1]Departments of Pathology, Xuanwu Hospital Capital Medical University, Beijing [*1]Department of Pathology, Xuanwu Hospital Capital Medical University, No. 45, Changchun Street, Xicheng District, Beijing 100053, China
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