The Medical College of Georgia at Augusta University, Augusta, GA, USA.
Department of Neurosurgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA.
J Neurooncol. 2022 Mar;157(1):187-195. doi: 10.1007/s11060-022-03961-5. Epub 2022 Feb 25.
Genetic analyses of gliomas have identified key molecular features that impact treatment paradigms beyond conventional histomorphology. Despite at-times lower grade histopathologic appearances, IDH-wildtype infiltrating gliomas expressing certain molecular markers behave like higher-grade tumors. For IDH-wildtype infiltrating gliomas lacking traditional features of glioblastoma, these markers form the basis for the novel diagnosis of diffuse astrocytic glioma, IDH-wildtype (wt), with molecular features of glioblastoma (GBM), WHO grade-IV (DAG-G). However, given the novelty of this approach to diagnosis, literature detailing the exact clinical, radiographic, and histopathologic findings associated with these tumors remain in development.
Data for 25 patients matching the DAG-G diagnosis were obtained from our institution's retrospective database. Information regarding patient demographics, treatment regimens, radiographic imaging, and genetic pathology were analyzed to determine association with clinical outcomes.
The initial radiographic findings, histopathology, and symptomatology of patients with DAG-G were similar to lower-grade astrocytomas (WHO grade 2/3). Overall survival (OS) and progression free survival (PFS) associated with our cohort, however, were similar to that of IDH-wt GBM, indicating a more severe clinical course than expected from other associated features (15.1 and 5.39 months respectively).
Despite multiple features similar to lower-grade gliomas, patients with DAG-G experience clinical courses similar to GBM. Such findings reinforce the need for biopsy and subsequent analysis of molecular features associated with any astrocytoma regardless of presenting characteristics.
对神经胶质瘤的基因分析确定了一些关键的分子特征，这些特征超越了传统的组织形态学，影响了治疗模式。尽管有时组织病理学表现为较低级别，但表达某些分子标志物的 IDH 野生型浸润性神经胶质瘤的行为与高级别肿瘤相似。对于缺乏胶质母细胞瘤传统特征的 IDH 野生型浸润性神经胶质瘤，这些标志物是新型弥漫性星形细胞瘤、IDH 野生型（wt）、具有胶质母细胞瘤（GBM）分子特征的诊断基础，WHO 分级-IV（DAG-G）。然而，鉴于这种诊断方法的新颖性，详细描述与这些肿瘤相关的临床、影像学和组织病理学发现的文献仍在不断发展。
从我们机构的回顾性数据库中获取了 25 名符合 DAG-G 诊断标准的患者的数据。分析了有关患者人口统计学、治疗方案、影像学和遗传学病理的信息，以确定与临床结果的关联。
DAG-G 患者的初始影像学表现、组织病理学和症状与低级别星形细胞瘤（WHO 分级 2/3）相似。然而，我们队列的总生存期（OS）和无进展生存期（PFS）与 IDH-wt GBM 相似，表明与其他相关特征相比，临床过程更为严重（分别为 15.1 和 5.39 个月）。
尽管有多种与低级别神经胶质瘤相似的特征，但 DAG-G 患者的临床过程与 GBM 相似。这些发现强调了无论表现特征如何，对任何星形细胞瘤进行活检和随后分析与分子特征相关的必要性。
