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原发性颅内胶质肉瘤:它真的是胶质母细胞瘤的一种变体吗?临床、放射学及生物分子特征的最新进展

Primary Intracranial Gliosarcoma: Is It Really a Variant of Glioblastoma? An Update of the Clinical, Radiological, and Biomolecular Characteristics.

作者信息

La Torre Domenico, Della Torre Attilio, Lo Turco Erica, Longo Prospero, Pugliese Dorotea, Lacroce Paola, Raudino Giuseppe, Romano Alberto, Lavano Angelo, Tomasello Francesco

机构信息

Department of Medical and Surgery Sciences, School of Medicine, AOU "Renato Dulbecco", University of Catanzaro, 88100 Catanzaro, Italy.

Humanitas, Istituto Clinico Catanese, 95045 Catania, Italy.

出版信息

J Clin Med. 2023 Dec 22;13(1):83. doi: 10.3390/jcm13010083.

Abstract

Gliosarcomas (GS) are sporadic malignant tumors classified as a Glioblastoma (GBM) variant with IDH-wild type phenotype. It appears as a well-circumscribed lesion with a biphasic, glial, and metaplastic mesenchymal component. The current knowledge about GS comes from the limited literature. Furthermore, recent studies describe peculiar characteristics of GS, such as hypothesizing that it could be a clinical-pathological entity different from GBM. Here, we review radiological, biomolecular, and clinical data to describe the peculiar characteristics of PGS, treatment options, and outcomes in light of the most recent literature. A comprehensive literature review of PubMed and Web of Science databases was conducted for articles written in English focused on gliosarcoma until 2023. We include relevant data from a few case series and only a single meta-analysis. Recent evidence describes peculiar characteristics of PGS, suggesting that it might be a specific clinical-pathological entity different from GBM. This review facilitates our understanding of this rare malignant brain tumor. However, in the future we recommend multi-center studies and large-scale metanalyses to clarify the biomolecular pathways of PGS to develop new specific therapeutic protocols, different from conventional GBM therapy in light of the new therapeutic opportunities.

摘要

胶质肉瘤(GS)是散发性恶性肿瘤,被归类为具有异柠檬酸脱氢酶(IDH)野生型表型的胶质母细胞瘤(GBM)变体。它表现为一个边界清晰的病变,具有双相、神经胶质和化生间充质成分。目前关于GS的知识来自有限的文献。此外,最近的研究描述了GS的特殊特征,例如推测它可能是一种不同于GBM的临床病理实体。在此,我们根据最新文献回顾放射学、生物分子和临床数据,以描述原发性胶质肉瘤(PGS)的特殊特征、治疗选择和结果。对PubMed和Web of Science数据库进行了全面的文献综述,以查找截至2023年用英文撰写的关于胶质肉瘤的文章。我们纳入了一些病例系列的相关数据,并且只纳入了一项荟萃分析。最近的证据描述了PGS的特殊特征,表明它可能是一种不同于GBM的特定临床病理实体。这篇综述有助于我们对这种罕见的恶性脑肿瘤的理解。然而,未来我们建议进行多中心研究和大规模荟萃分析,以阐明PGS的生物分子途径,从而根据新的治疗机会开发不同于传统GBM治疗的新的特异性治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/84b0/10779593/e343f21dfea3/jcm-13-00083-g001.jpg

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