IDH 野生型 2 级弥漫性星形细胞瘤：分子亚群内的预后因素和治疗影响。
IDH wild-type grade 2 diffuse astrocytomas: prognostic factors and impact of treatments within molecular subgroups.
机构信息
Department of Neuro-Oncology, University and City of Health and Science Hospital, Turin, Italy.
Department of Neurology, Castelfranco Veneto and Brain Tumor Board Treviso Hospital, Italy.
出版信息
Neuro Oncol. 2022 May 4;24(5):809-820. doi: 10.1093/neuonc/noab239.
BACKGROUND
Prognostic factors and role of treatments are not well known in isocitrate dehydrogenase (IDH) wild-type (wt) grade 2 astrocytomas. The aim of this study was to define in these tumors clinical features, molecular characteristics, and prognostic factors, with particular focus on molecular subgroups defined by cIMPACT-NOW update 3.
METHODS
We analyzed 120 patients with confirmed diagnosis of grade 2 IDHwt astrocytoma according to WHO 2016, collected from seven Italian centers between 1999 and 2017.
RESULTS
Median PFS and OS of the whole cohort were 18.9 and 32.6 months. Patients older than 40 years and patients with modest contrast enhancement on MRI had a shorter PFS and OS. Gross total resection yielded superior PFS and OS over non-gross total resection. PFS and OS of patients with either pTERT mutation or EGRF amplification were significantly shorter. The prognostic value of age, contrast enhancement on MRI, and extent of surgery was different within the molecular subgroups. Gross total resection was associated with increased PFS (not reached versus 14 months, p = 0.023) and OS (117.9 versus 20 months, p = 0.023) in patients without EGFR amplification, and with increased OS in those without pTERT mutation (NR vs 53.7 months, p = 0.05). Conversely, for patients with EGFR amplification or pTERT mutation, gross total resection did not yield a significant survival benefit.
CONCLUSION
Patients without EGFR amplification and pTERT mutation could be observed after gross total resection.
背景
异柠檬酸脱氢酶（IDH）野生型（wt）2 级星形细胞瘤的预后因素和治疗作用尚不清楚。本研究旨在确定这些肿瘤的临床特征、分子特征和预后因素，特别关注 cIMPACT-NOW 更新 3 定义的分子亚组。
方法
我们分析了 120 例经 WHO 2016 确诊的 IDHwt 2 级星形细胞瘤患者，这些患者来自 2017 年之前七个意大利中心的 1999 年至 2017 年的数据。
结果
全队列的中位无进展生存期（PFS）和总生存期（OS）分别为 18.9 和 32.6 个月。年龄大于 40 岁和 MRI 中度增强的患者 PFS 和 OS 更短。大体全切除的 PFS 和 OS 优于非大体全切除。存在 pTERT 突变或 EGRF 扩增的患者 PFS 和 OS 明显更短。年龄、MRI 增强程度和手术范围的预后价值在分子亚组中有所不同。在没有 EGFR 扩增的患者中，大体全切除与 PFS（未达到 vs 14 个月，p = 0.023）和 OS（117.9 vs 20 个月，p = 0.023）的改善相关，而在没有 pTERT 突变的患者中，与 OS 的改善相关（NR vs 53.7 个月，p = 0.05）。相反，对于存在 EGFR 扩增或 pTERT 突变的患者，大体全切除并不能带来显著的生存获益。
结论
没有 EGFR 扩增和 pTERT 突变的患者可以在大体全切除后进行观察。
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