Department of Pathology and Genomic Medicine, The Methodist Hospital, 6565 Fannin St, M227, Houston, TX 77030, USA.
Curr Treat Options Oncol. 2012 Dec;13(4):417-36. doi: 10.1007/s11864-012-0210-8.
Diffuse gliomas are the most common primary brain tumors, with glioblastoma (GBM) encompassing more than 50 % of all cases. Despite aggressive therapy, patients nearly always succumb to their disease and the survival for patients with GBM is approximately 1 year. During past years, numerous scientific contributions have reshaped the field of neuro-oncology and neuropathology. A series of molecular discoveries have shed light on new pathogenic mechanisms, as well as new prognostic and predictive biomarkers with clinical relevance. The current World Health Organization (WHO) classification system is solely based on morphologic criteria; however, there is accumulated evidence that tumors with similar histology have distinct molecular signatures with a clinically significant impact on treatment response and survival. Molecular markers and signatures could be incorporated into the glioma classification and grading system to mirror the clinical outcomes. Additionally, molecular markers could lead to a redefinition of currently controversial entities, such as mixed oligoastrocytomas. Newly discovered molecular alterations also have the potential to become targets for future drug development. Despite tremendous progress in the past decade, therapeutic progress for diffuse gliomas has been slow. A further understanding of glioma biology, in concert with well-designed clinical trials, is necessary to identify more putative molecular biomarkers and unravel the mysteries in the pathogenic mechanisms that trigger this menacing disease.
弥漫性神经胶质瘤是最常见的原发性脑肿瘤,其中胶质母细胞瘤(GBM)占所有病例的 50%以上。尽管采用了积极的治疗方法,但几乎所有患者最终都会死于该疾病,GBM 患者的生存时间约为 1 年。在过去的几年中,大量的科学贡献改变了神经肿瘤学和神经病理学领域。一系列的分子发现揭示了新的发病机制,以及具有临床相关性的新的预后和预测生物标志物。目前的世界卫生组织(WHO)分类系统仅基于形态学标准;然而,有越来越多的证据表明,具有相似组织学的肿瘤具有不同的分子特征,对治疗反应和生存有明显的临床影响。分子标志物和特征可以纳入神经胶质瘤的分类和分级系统,以反映临床结果。此外,分子标志物可以重新定义目前存在争议的实体,如混合性少突胶质细胞瘤。新发现的分子改变也有可能成为未来药物开发的靶点。尽管在过去十年中取得了巨大进展,但弥漫性神经胶质瘤的治疗进展缓慢。为了确定更多可能的分子生物标志物并揭示导致这种威胁生命的疾病的发病机制中的奥秘,有必要进一步了解神经胶质瘤的生物学,并结合精心设计的临床试验。