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原发性胶质肉瘤的人口统计学、影像学、分子及临床特征以及与胶质母细胞瘤的差异

Demographic, radiographic, molecular and clinical characteristics of primary gliosarcoma and differences to glioblastoma.

作者信息

Pierscianek Daniela, Ahmadipour Yahya, Michel Anna, Rauschenbach Laurèl, Darkwah Oppong Marvin, Deuschl Cornelius, Kebir Sied, Wrede Karsten H, Glas Martin, Stuschke Martin, Sure Ulrich, Jabbarli Ramazan

机构信息

Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany; German Cancer Consortium, Partner Site University Hospital Essen, Essen, Germany.

Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany; German Cancer Consortium, Partner Site University Hospital Essen, Essen, Germany.

出版信息

Clin Neurol Neurosurg. 2021 Jan;200:106348. doi: 10.1016/j.clineuro.2020.106348. Epub 2020 Nov 1.

DOI:10.1016/j.clineuro.2020.106348
PMID:33158630
Abstract

OBJECTIVE

Gliosarcoma (GSC) is a rare histological variant of glioblastoma (GBM). Due to limited evidence regarding clinical, genetic and radiographic characteristics of GSC, this study aimed to analyze independent outcome predictors of GSC, and to address the differences between GSC and GBM concerning the baseline characteristics and patients' survival.

METHODS

Patients treated between 2001 and 2018 for the diagnosis of GBM and GSC were included in this study. Patients' records were reviewed for demographic, clinical, genetic and radiographic characteristics. Univariate, multivariate and propensity score matched analyses were performed.

RESULTS

In the GSC sub-cohort (N = 56), patients' age, preoperative clinical status, midline tumor location and tumor size were found to be independently associated with overall survival. As compared to GBM individuals (N = 1249), a temporal location (p = 0.002), presence of eccentric tumor cysts (p < 0.001), a higher ratio of TP53 staining (p = 0.002) and a lower ratio of GFAP staining (p = 0.005) were characteristic for GSC. The diagnosis of GSC was associated with a poorer survival (p = 0.002) independently of the patients' age, sex, clinical status and extent of resection, However, this association was no more significant, when enhancing the multivariate analysis with molecular-genetic characteristics (IDH1 mutation and MGMT promotor methylation status).

DISCUSSION

Certain radiographic and molecular-genetic patterns present the distinct characteristics of GSC. There is an association between the diagnosis of GSC and a poorer outcome. This difference might be linked to different genetic alterations in GBM and GSC. Prospective studies are needed to further elucidate the characteristics of GSC and develop targeted treatment approaches for this rare variant.

摘要

目的

胶质肉瘤(GSC)是胶质母细胞瘤(GBM)一种罕见的组织学变体。由于关于GSC临床、遗传和影像学特征的证据有限,本研究旨在分析GSC的独立预后预测因素,并探讨GSC与GBM在基线特征和患者生存方面的差异。

方法

本研究纳入了2001年至2018年期间因诊断为GBM和GSC而接受治疗的患者。回顾患者记录以获取人口统计学、临床、遗传和影像学特征。进行单因素、多因素和倾向评分匹配分析。

结果

在GSC亚组(N = 56)中,发现患者年龄、术前临床状态、肿瘤位于中线以及肿瘤大小与总生存期独立相关。与GBM患者(N = 1249)相比,GSC的特征为肿瘤位于颞叶(p = 0.002)、存在偏心肿瘤囊肿(p < 0.001)、TP53染色比例较高(p = 0.002)和GFAP染色比例较低(p = 0.005)。GSC的诊断与较差的生存率相关(p = 0.002),且独立于患者的年龄、性别、临床状态和切除范围。然而,当在多因素分析中加入分子遗传学特征(IDH1突变和MGMT启动子甲基化状态)时,这种关联不再显著。

讨论

某些影像学和分子遗传学模式呈现出GSC的独特特征。GSC的诊断与较差的预后相关。这种差异可能与GBM和GSC中不同的基因改变有关。需要进行前瞻性研究以进一步阐明GSC的特征并为这种罕见变体开发靶向治疗方法。

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