Department of Hygiene, Epidemiology and Medical Statistics, School of Medicine, National and Kapodistrian University of Athens, 75 Mikras Asias Str, 11527, Athens, Greece.
Department of Pediatric Hematology-Oncology, "Pan. & Agl. Kyriakou" Children's Hospital, Thivon 18, 11527, Athens, Greece.
J Neurooncol. 2018 Jun;138(2):341-349. doi: 10.1007/s11060-018-2802-z. Epub 2018 Feb 20.
Gliomatosis cerebri (GC) comprises a rare widespread infiltrating growth pattern of diffuse gliomas. We explored the incidence patterns and survival rates of GC in a population-based registration sample from the Surveillance, Epidemiology and End, Results database (1973-2012). GC cases (n = 176) were identified based on their International Classification of Diseases in Oncology (ICD-O-3) morphology code (9381). We calculated age-adjusted incidence rates (AIR) and evaluated temporal trends. Survival was assessed with Kaplan-Meier curves and Cox regression models. The annual AIR of GC was 0.1/million. We noted increasing trends in the preceding registration years (1973-2002; annually, + 7%) and a tendency of clinical/radiological approaches to substitute the gold-standard histological assessment for diagnosis. GC was diagnosed in the entire age spectrum (range 1-98 years), but higher incidence rates (0.43/million) were noted among the elderly (≥ 65 years). A slight male preponderance was identified (male-to-female ratio: 1.4). Median overall survival was 9 months with a 5 year survival rate of 18%. Increasing age, primary tumor location not restricted to the cerebral hemispheres and rural residence at diagnosis were identified as negative prognostic factors, whereas receipt of radiotherapy, surgical treatment, race and method of diagnosis were not associated with outcome. This first comprehensive overview of GC epidemiology exemplifies the rarity of the disease, provides evidence for male preponderance and increased incidence among the elderly and shows lower survival rates compared to the published single center reports. Expansion of registration to histological and molecular characteristics would allow emergence of clinical prognostic factors at the population level.
脑胶质瘤病（Gliomatosis cerebri，GC）是一种罕见的弥漫性胶质瘤弥漫浸润生长模式。我们在 Surveillance, Epidemiology and End Results（SEER）数据库（1973-2012 年）的人群登记样本中探索了 GC 的发病模式和生存率。根据国际肿瘤疾病分类（ICD-O-3）形态学代码（9381）确定 GC 病例（n=176）。我们计算了年龄调整发病率（age-adjusted incidence rate，AIR）并评估了时间趋势。使用 Kaplan-Meier 曲线和 Cox 回归模型评估生存情况。GC 的年 AIR 为 0.1/百万人。我们注意到在之前的登记年份（1973-2002 年）中呈上升趋势（每年增加 7%），并且临床/影像学方法有取代金标准组织学评估进行诊断的趋势。GC 诊断发生在整个年龄谱（范围 1-98 岁）中，但在老年人（≥65 岁）中发病率较高（0.43/百万人）。存在轻微的男性优势（男女性别比：1.4）。总体中位生存时间为 9 个月，5 年生存率为 18%。我们确定了年龄增长、原发肿瘤位置不限于大脑半球和诊断时居住在农村地区是负预后因素，而放疗、手术治疗、种族和诊断方法与结局无关。这是对 GC 流行病学的首次全面概述，说明了该疾病的罕见性，提供了男性优势和老年人群发病率增加的证据，并显示与已发表的单中心报告相比生存率较低。扩大登记范围以纳入组织学和分子特征将允许在人群水平上出现临床预后因素。
