Dr. Senckenberg Institute of Neurooncology, Goethe University, Frankfurt am Main, Germany,
University Cancer Center Frankfurt (UCT), Goethe University, Frankfurt am Main, Germany,
Oncology. 2021;99(4):215-224. doi: 10.1159/000512562. Epub 2021 Jan 20.
Gliomatosis cerebri (GC) is defined by diffuse, widespread glial tumor growth affecting three or more cerebral lobes. Previous studies in gliomas found no distinct histological or molecular GC subtype, yet the presence of GC is associated with worse median overall survival (OS). Here, we explored whether differing therapeutic strategies in first-line treatment could account for this.
From our University Cancer Center database, 47 patients with histological diagnosis of WHO grade II or III glioma and GC imaging pattern were identified. GC criteria were confirmed by independent review. Patients with WHO grade II or III glioma with non-GC pattern served as control cohort (n = 343).
Within the GC patient cohort, lower WHO grade, mutated isocitrate dehydrogenase 1 (IDH1) status, and absence of contrast enhancement were associated with better OS. Compared to the control cohort, patients with GC had significantly shorter OS independent of histological diagnosis or IDH1 mutation status. Patients with GC preferentially received chemotherapy alone (62 vs. 18%), and less frequently radiochemotherapy (21 vs. 27%). OS was significantly shorter in the GC cohort compared to the non-GC cohort both for chemotherapy (3.9 vs. 7.6 years, p = 0.0085) and for combined radiochemotherapy (1.1 vs. 8.4 years, p < 0.0001). However, when only patients who received biopsy plus chemotherapy were analyzed, the differences lost statistical significance (3.5 vs. 6.6 years, p = 0.196).
We found major differences in the selection of first-line therapies of GC versus non-GC patients. Our results suggest that these differences may partly account for the worse prognosis of GC patients.
脑胶质瘤病(Gliomatosis cerebri,GC)是指弥漫性、广泛分布的胶质肿瘤生长,累及三个或更多脑叶。先前的胶质瘤研究未发现明确的组织学或分子 GC 亚型,但 GC 的存在与中位总生存期(Overall survival,OS)较差相关。在此,我们探讨了一线治疗中不同的治疗策略是否可以解释这一点。
从我们的大学癌症中心数据库中,确定了 47 名组织学诊断为 WHO 二级或三级胶质瘤且具有 GC 影像学模式的患者。通过独立审查确认 GC 标准。WHO 二级或三级胶质瘤且非 GC 模式的患者作为对照队列(n = 343)。
在 GC 患者队列中,较低的 WHO 分级、突变型异柠檬酸脱氢酶 1(IDH1)状态和无对比增强与更好的 OS 相关。与对照队列相比,GC 患者的 OS 明显更短,与组织学诊断或 IDH1 突变状态无关。与对照组相比,GC 患者更倾向于单独接受化疗(62% vs. 18%),而较少接受放化疗(21% vs. 27%)。与非 GC 队列相比,GC 队列接受化疗(3.9 年 vs. 7.6 年,p = 0.0085)和联合放化疗(1.1 年 vs. 8.4 年,p < 0.0001)的 OS 均显著缩短。然而,当仅分析接受活检加化疗的患者时,差异失去统计学意义(3.5 年 vs. 6.6 年,p = 0.196)。
我们发现 GC 与非 GC 患者在一线治疗选择上存在显著差异。我们的结果表明,这些差异可能部分解释了 GC 患者预后较差的原因。