Scott J N, Rewcastle N B, Brasher P M, Fulton D, Hagen N A, MacKinnon J A, Sutherland G, Cairncross J G, Forsyth P
Department of Clinical Neurosciences, University of Calgary.
Can J Neurol Sci. 1998 Aug;25(3):197-201. doi: 10.1017/s0317167100034016.
Long-term glioblastoma multiforme survivors (LTGBMS) are uncommon. The frequency which these occur in an unselected population and factors which produce these unusually long survivors are unknown.
To determine in a population-based study 1) the frequency of LTGBMS in a population and 2) identify which patient, treatment or tumor characteristics would predict which glioblastoma (GBM) patient would become a LTGBMS.
The Alberta Cancer Registry was used to identify all patients diagnosed with GBM in southern Alberta between 1/1/75-12/31/91. Patient charts were reviewed and histology re-examined by a blinded neuropathologist. LTGBMS were defined as GBM patients surviving > or = 3 years after diagnosis. Each LTGBMS was compared to three age-, gender-, and year of diagnosis-matched controls to compare patient, treatment, and tumor factors to GBM patients without long-term survival.
There were 279 GBMs diagnosed in the study period. Five (1.8%) survived > or = three years (range, 3.2-15.8 years). Seven additional long-term survivors, who carried a diagnosis of GBM, were excluded after neuropathologic review; the most common revised diagnosis was malignant oligodendroglioma. LTGBMS (avg. age = 45 years) were significantly younger when compared to all GBM patients (avg. age = 59 years, p = 0.0001) diagnosed in the study period. LTGBMS had a higher KPS at diagnosis (p = 0.001) compared to controls. Tumors from LTGBMS tended to have fewer mitoses and a lower Ki-67 cellular proliferative index compared to controls. Radiation-induced dementia was common and disabling in LTGBMS.
These data highlight the dismal prognosis for GBM patients who have both a short median survival and very small chance (1.8%) of long-term survival. The LTGBMS were younger, had a higher performance status, and their tumors tended to proliferate less rapidly than control GBM patients. When long-term survival does occur it is often accompanied by severe treatment-induced dementia.
长期多形性胶质母细胞瘤幸存者（LTGBMS）并不常见。在未经过挑选的人群中这些幸存者出现的频率以及产生这些超长生存期幸存者的因素尚不清楚。
在一项基于人群的研究中确定1）人群中LTGBMS的频率，以及2）确定哪些患者、治疗或肿瘤特征可以预测哪些胶质母细胞瘤（GBM）患者会成为LTGBMS。
使用艾伯塔癌症登记处来识别1975年1月1日至1991年12月31日期间在艾伯塔省南部被诊断为GBM的所有患者。回顾患者病历并由一位不知情的神经病理学家重新检查组织学。LTGBMS被定义为诊断后存活≥3年的GBM患者。将每位LTGBMS与三名年龄、性别和诊断年份匹配的对照进行比较，以比较患者、治疗和肿瘤因素与无长期生存的GBM患者。
在研究期间共诊断出279例GBM。五例（1.8%）存活≥三年（范围为3.2 - 15.8年）。在神经病理学复查后，另外七名诊断为GBM的长期幸存者被排除；最常见的修正诊断是恶性少突胶质细胞瘤。与研究期间诊断的所有GBM患者（平均年龄 = 59岁，p = 0.0001）相比，LTGBMS（平均年龄 = 45岁）明显更年轻。与对照组相比，LTGBMS在诊断时的KPS更高（p = 0.001）。与对照组相比，LTGBMS的肿瘤往往有较少的有丝分裂和较低的Ki-67细胞增殖指数。放射性痴呆在LTGBMS中很常见且会导致残疾。
这些数据凸显了GBM患者预后不佳，其生存期中位数短且长期生存的机会非常小（1.8%）。LTGBMS患者更年轻，具有更高的体能状态，并且他们的肿瘤增殖速度往往比对照GBM患者慢。当确实发生长期生存时，往往伴随着严重的治疗引起的痴呆。
