Goryaynov S A, Gol'dberg M F, Golanov A V, Zolotova S V, Shishkina L V, Ryzhova M V, Pitskhelauri D I, Zhukov V Yu, Usachev D Yu, Belyaev A Yu, Kondrashov A V, Shurkhay V A, Potapov A A
Burdenko Neurosurgical Institute, Moscow, Russia.
Burdenko Neurosurgical Institute, Moscow, Russia; Sechenov First Moscow State Medical University, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2017;81(3):5-16. doi: 10.17116/neiro20178135-16.
The median overall survival of glioblastoma patients is about 15 months. Only a small number of patients survive 3 years. The factors of a favorable prognosis for the 'longevity phenomenon' in glioblastoma patients are not fully understood.
to determine the occurrence rate of long-living patients with glioblastomas, identify clinical predictors of a favorable prognosis, and identify the presence and prognostic significance of an IDH1 mutation.
Among 1494 patients operated on for glioblastoma at the Burdenko Neurosurgical Institute from 2007 to 2012, there were 84 (5.6%) patients who lived more than 3 years after primary surgery. In all the cases, histological specimens were reviewed, and immunohistochemical detection of a mutant IDH1 protein was performed. Overall survival was calculated from the time of first surgery to the date of the last consultation or death, and the recurrence-free period was calculated from the time of first surgery to MRI-verified tumor progression.
The median age of long-living patients with glioblastoma was 45 years (19-65 years). All tumors were located supratentorially. The median Karnofsky performance status score at the time of surgery was 80 (range, 70-100). All patients underwent microsurgical resection of the tumor, followed by chemoradiotherapy. The median recurrence-free period was 36 months (5-98 months). Overall survival of 48, 60, and 84 months was achieved in 23, 15 and 6% of patients, respectively. Among 49 specimens available for the IDH1 analysis, 14 (28.6%) specimens had a mutant protein. There was no significant difference in survival rates in patients with positive and negative results for IDH1 (44.1 vs. 40.8 months; p>0.05).
The significance of various factors that may be predictors of a favorable course of the disease is discussed in the literature. This work is the first part of analysis of prognostically significant factors positively affecting overall survival of glioblastoma patients. In our series, the predictors of a favorable prognosis for long-living patients with the verified diagnosis of glioblastoma were as follows: young age, the supratentorial location of the tumor, a high Karnofsky score before surgery, and tumor resection. In our series, we used immunohistochemical tests and found no prognostic significance of the IDH1 gene mutation; further analysis will require application of direct sequencing. We plan to study other morphological and molecular genetic features of tumors, which explain prolonged survival of glioblastoma patients, as well as the role of various types of combined chemoradiation treatment.
胶质母细胞瘤患者的中位总生存期约为15个月。只有少数患者能存活3年。胶质母细胞瘤患者“长寿现象”的有利预后因素尚未完全明确。
确定胶质母细胞瘤长期存活患者的发生率，识别有利预后的临床预测因素，并确定异柠檬酸脱氢酶1（IDH1）突变的存在及其预后意义。
2007年至2012年在布尔坚科神经外科研究所接受胶质母细胞瘤手术的1494例患者中，有84例（5.6%）在初次手术后存活超过3年。所有病例均复查了组织学标本，并进行了突变型IDH1蛋白的免疫组化检测。总生存期从首次手术时间计算至最后一次会诊或死亡日期，无复发生存期从首次手术时间计算至MRI证实的肿瘤进展。
胶质母细胞瘤长期存活患者的中位年龄为45岁（19 - 65岁）。所有肿瘤均位于幕上。手术时卡诺夫斯基功能状态评分中位数为80（范围70 - 100）。所有患者均接受了肿瘤显微手术切除，随后进行放化疗。中位无复发生存期为36个月（5 - 98个月）。分别有23%、15%和6%的患者总生存期达到48、60和84个月。在可进行IDH1分析的49份标本中，14份（28.6%）标本有突变蛋白。IDH1检测结果为阳性和阴性的患者生存率无显著差异（44.1个月对40.8个月；p>0.05）。
文献中讨论了可能是疾病良好病程预测因素的各种因素的意义。这项工作是对积极影响胶质母细胞瘤患者总生存期的预后显著因素分析的第一部分。在我们的系列研究中，经确诊的胶质母细胞瘤长期存活患者的有利预后预测因素如下：年轻、肿瘤位于幕上、手术前卡诺夫斯基评分高以及肿瘤切除。在我们的系列研究中，我们使用免疫组化检测，发现IDH1基因突变无预后意义；进一步分析需要应用直接测序。我们计划研究肿瘤的其他形态学和分子遗传学特征，这些特征可解释胶质母细胞瘤患者的长期存活情况，以及各种联合放化疗治疗的作用。
