Department of Neurosurgery, Ludwig-Maximilians-University Munich, Marchioninistr. 15, 81377, Munich, Germany.
German Cancer Consortium (DKTK), Partner Site Munich, and German Cancer Research Center (DKFZ), Heidelberg, Germany.
J Neurooncol. 2018 Sep;139(3):671-678. doi: 10.1007/s11060-018-2912-7. Epub 2018 May 28.
Transcriptional activating mutations in the promoter region of the telomerase reverse transcriptase (TERT) gene occur at high frequency in various types of solid tumors and have also been reported for meningiomas. Especially for atypical and anaplastic meningiomas, the prognostic relevance of TERT promoter mutation is yet unclear. The present study aimed to analyze the frequency of TERT promoter mutation and define its long-term prognostic significance beyond clinical and histological factors in a cohort of meningiomas WHO grade II and III.
Patients undergoing surgical resection of aggressive meningiomas were included. Analysis for C228T and C250T mutation in the TERT promoter region was performed using PCR method. Patients were stratified into two groups (TERT mutated vs. TERT wild type). Univariate analysis was conducted using molecular and histological factors.
87 patients with atypical (N = 72) and anaplastic meningiomas (N = 15) were included in the study. TERT promoter region was found to be mutated in 4 WHO grade II and 2 WHO grade III meningiomas. TERT promoter mutation was associated with shorter progression free survival than TERT wild type meningiomas (median PFS 12.5 vs. 26 months, p = .004). In the univariate analysis, TERT promoter mutation had a strong prognostic value on overall survival (p = .009) and progression free survival.
Presence of TERT promoter mutation is associated with shorter progression free survival and overall survival in meningiomas WHO grade II and III. In these tumors, TERT promoter mutation should be considered as a clinically relevant prognostic factor to identify high risk patients.
端粒酶逆转录酶（TERT）基因启动子区域的转录激活突变在各种实体肿瘤中高频发生，也有报道见于脑膜瘤。特别是对于非典型和间变性脑膜瘤，TERT 启动子突变的预后相关性尚不清楚。本研究旨在分析 TERT 启动子突变的频率，并在 II 级和 III 级脑膜瘤患者队列中定义其在临床和组织学因素之外的长期预后意义。
纳入接受侵袭性脑膜瘤手术切除的患者。采用 PCR 法分析 TERT 启动子区域的 C228T 和 C250T 突变。患者分为两组（TERT 突变型与 TERT 野生型）。采用单变量分析方法分析分子和组织学因素。
研究纳入了 87 例非典型性（N=72）和间变性脑膜瘤（N=15）患者。发现 4 例 II 级和 2 例 III 级脑膜瘤存在 TERT 启动子区域突变。与 TERT 野生型脑膜瘤相比，TERT 启动子突变与较短的无进展生存期相关（中位 PFS 12.5 与 26 个月，p=0.004）。单变量分析显示，TERT 启动子突变对总生存期（p=0.009）和无进展生存期具有强烈的预后价值。
TERT 启动子突变与 II 级和 III 级脑膜瘤的无进展生存期和总生存期较短相关。在这些肿瘤中，TERT 启动子突变应被视为一种具有临床相关性的预后因素，以识别高危患者。
