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新诊断原发性胶质母细胞瘤青少年和年轻成人患者的结局和分子特征:奥地利神经肿瘤学会(SANO)的一项研究。

Outcome and molecular characteristics of adolescent and young adult patients with newly diagnosed primary glioblastoma: a study of the Society of Austrian Neurooncology (SANO).

机构信息

Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria.

出版信息

Neuro Oncol. 2013 Jan;15(1):112-21. doi: 10.1093/neuonc/nos283. Epub 2012 Dec 7.

Abstract

BACKGROUND

Young age is a favorable prognostic factor for patients with glioblastoma multiforme (GBM). We reviewed the outcomes and molecular tumor characteristics of adolescent and young adult patients with GBM treated in 2 Austrian centers.

PATIENTS AND METHODS

Data on patients with histologically proven primary GBM diagnosed from 18 through 40 years of age were retrospectively analyzed. All patients were treated with standard first-line therapy. The primary end points were overall survival (OS) and time to progression (TTP). IDH1-R132H mutation status was analyzed using immunohistochemistry, and MGMT promoter methylation was assessed using methylation-specific polymerase chain reaction.

RESULTS

We included 70 patients (36 men and 34 women) with a median age of 33 years. IDH1-R132H mutations were detected in 22 (39.3%) of 56 cases and MGMT promoter methylation in 33 (61.1%) of 54 cases with available tissue samples. In patients with wild-type IDH, median TTP was 8.2 months and median OS was 24 months, compared with 18 months and 44 months, respectively, observed in patients with mutated IDH. Neither IDH1 nor MGMT status showed a statistically significant association with TTP or OS. Of note, the social and economical situation of the young patients with GBM was alarming, because only 17% succeeded in staying employed after receiving the diagnosis.

CONCLUSIONS

We found a high frequency of IDH1 mutations and MGMT promoter methylation among young adult patients with primary GBM that may contribute to the generally favorable outcome associated with young age. The social and economic coverage of patients with glioma remains an unsolved socio-ethical problem.

摘要

背景

年轻是胶质母细胞瘤(GBM)患者的有利预后因素。我们回顾了在奥地利的 2 个中心接受治疗的青少年和年轻成人胶质母细胞瘤患者的结果和分子肿瘤特征。

患者和方法

回顾性分析了经组织学证实的 18 至 40 岁原发性 GBM 患者的数据。所有患者均接受标准一线治疗。主要终点是总生存期(OS)和无进展生存期(TTP)。使用免疫组织化学检测 IDH1-R132H 突变状态,使用甲基化特异性聚合酶链反应评估 MGMT 启动子甲基化。

结果

我们纳入了 70 例患者(36 名男性和 34 名女性),中位年龄为 33 岁。在 56 例可获得组织样本的病例中,检测到 22 例(39.3%)IDH1-R132H 突变,33 例(61.1%)MGMT 启动子甲基化。在 IDH 野生型患者中,中位 TTP 为 8.2 个月,中位 OS 为 24 个月,而 IDH 突变型患者的 TTP 和 OS 分别为 18 个月和 44 个月。IDH1 或 MGMT 状态均与 TTP 或 OS 无显著相关性。值得注意的是,患有 GBM 的年轻患者的社会和经济状况令人担忧,因为只有 17%的患者在确诊后成功就业。

结论

我们发现,年轻的原发性 GBM 患者中 IDH1 突变和 MGMT 启动子甲基化的频率很高,这可能是与年轻相关的总体预后较好的原因。胶质母细胞瘤患者的社会和经济保障仍然是一个未解决的社会伦理问题。

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