Adriaensen M E A P M, Schaefer-Prokop C M, Stijnen T, Duyndam D A C, Zonnenberg B A, Prokop M
Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
Eur J Neurol. 2009 Jun;16(6):691-6. doi: 10.1111/j.1468-1331.2009.02567.x. Epub 2009 Feb 19.
To investigate the prevalence of subependymal giant cell ependymomas (SEGA) in patients with tuberous sclerosis complex (TSC).
We performed a retrospective cross-sectional study in a cohort of 285 patients with known TSC. Institutional review board approval was obtained. We included all 214 TSC-patients who had received a contrast-enhanced computed tomography (CT) scan of the brain. The most recent scan was evaluated for SEGA and presence of hydrocephalus. Additionally, a literature search was performed, and pooled estimates of SEGA prevalence in TSC were calculated. We used descriptive statistics, two sample t-test, chi-squared-test, and meta-analysis as appropriate.
Computed tomography showed radiological evidence of SEGA in 43 of the 214 TSC-patients (20%); 23 of 105 men (22%) and 20 of 109 women (18%; P = .52). Average maximum tumor size was 11.4 mm (range, 4-29 mm). Patients with SEGA (mean, 31 years; range, 16-58 years) were on average younger than patients without SEGA (mean, 37 years; range, 10-72 years; P = 0.007). No association between tumor size and patient age was detected. Nine patients had bilateral SEGA. Hydrocephalus was present in six of the 43 patients (14%). Meta-analysis of reported prevalence and our current study showed that studies using radiological evidence to diagnose SEGA gave a higher pooled estimate of the prevalence of SEGA in TSC (0.16; 95% CI: 0.12, 0.21) than studies using mainly histopathological evidence of SEGA (0.09; 95% CI: 0.07, 0.12).
In our cohort, CT demonstrated evidence of SEGA in 20% of TSC-patients. Prevalence of SEGA in TSC is higher in studies using radiological evidence to diagnose SEGA than in studies using histopathological evidence.
研究结节性硬化症（TSC）患者室管膜下巨细胞型室管膜瘤（SEGA）的患病率。
我们对285例已知患有TSC的患者进行了一项回顾性横断面研究。获得了机构审查委员会的批准。我们纳入了所有214例接受过脑部增强计算机断层扫描（CT）的TSC患者。对最近的扫描结果进行SEGA及脑积水情况评估。此外，进行了文献检索，并计算了TSC中SEGA患病率的汇总估计值。我们酌情使用了描述性统计、两样本t检验、卡方检验和荟萃分析。
CT显示214例TSC患者中有43例（20%）有SEGA的影像学证据；105名男性中有23例（22%），109名女性中有20例（18%；P = 0.52）。肿瘤平均最大尺寸为11.4毫米（范围4 - 29毫米）。患有SEGA的患者（平均年龄31岁；范围16 - 58岁）平均比未患SEGA的患者年轻（平均年龄37岁；范围10 - 72岁；P = 0.007）。未检测到肿瘤大小与患者年龄之间的关联。9例患者有双侧SEGA。43例患者中有6例（14%）存在脑积水。对已报道的患病率与我们当前研究进行的荟萃分析表明，与主要使用SEGA组织病理学证据的研究（0.09；95%置信区间：0.07，0.12）相比，使用影像学证据诊断SEGA时，TSC中SEGA患病率的汇总估计值更高（0.16；95%置信区间：0.12，0.21）。
在我们的队列中，CT显示20%的TSC患者有SEGA的证据。与使用组织病理学证据的研究相比，使用影像学证据诊断SEGA时，TSC中SEGA的患病率更高。
