结节性硬化症12例临床影像分析  

Clinical image analysis of 12 patients with tuberous sclerosis complex

作　　者：王雅菁 卜顺林 闵钢 范小丽 陈明亮 WANG Yajing;BU Shunlin;MIN Gang;FAN Xiaoli;CHEN Mingliang(Department of Radiology,Affiliated Nanjing Jiangbei Hospital of Nantong University Xinglin College,Nanjing,Jiangsu 210048,China)

机构地区：[1]南通大学杏林学院附属南京江北医院放射科,江苏南京210048

出　　处：《中国临床研究》2023年第11期1708-1712,1717,共6页Chinese Journal of Clinical Research

摘　　要：目的分析结节硬化症(TSC)的临床、CT和MRI的特征,以提高对该疾病多器官损害的认识。方法回顾分析2015年9月至2020年6月12例于南京江北医院临床确诊TSC患者的临床、CT和MRI特征。结果TSC累及多器官,有不同的影像学改变(例数统计可出现重叠)。中枢神经系统:典型的室管膜下结节9例,其中钙化结节7例,非钙化结节2例;皮质及皮质下结节5例,脑白质异常信号改变3例,室管膜下巨细胞星形细胞瘤1例。腹部:双肾大小不等的血管平滑肌脂肪瘤8例,其中2例伴有肿瘤内出血;肝脏多发性血管平滑肌瘤1例。胸部:肺淋巴管平滑肌瘤病2例,心脏横纹肌瘤1例。骨骼:骨骼多发结节样、斑片状骨质硬化1例。结论分析TSC多系统肿瘤的临床影像学征象,为TSC的临床诊断提供依据。Objective To analyze the clinical,CT and MRI characteristics of tuberous sclerosis complex(TSC)to raise awareness of multiple organ damage in this disease.Methods The clinical,CT and MRI characteristics of 12 cases of clinically confirmed TSC in Nanjing Jiangbei Hospital from September 2015 to June 2020 were retrospectively analyzed.Results TSC involved multiple organs and showed different changes in clinical imaging(the count of cases may overlap).In central nervous system,there were 9 cases of typical subependymal nodule,including 7 calcified nodules and 2 non-calcified nodules;5 cases of cortical and subcortical nodule,3 cases of white matter signal change,1 case of subependymal giant cell astrocytoma.In abdomen,there were 8 cases of angiomyolipoma of different sizes in both kidneys(of which 2 cases were accompanied by intratumoral hemorrhage)and 1 case of multiple hepatic angiomyolipoma.In chest,there were 2 cases of pulmonary lymphangioleiomyomatosis and 1 case of cardiac rhabdomyoma.In bone,there was 1 case of multiple nodular and patchy osteosclerosis.Conclusion The analysis of clinical imaging features of TSC multi-system tumor is helpful for the diagnosis of TSC.

关 键 词：结节性硬化症 多器官肿瘤 电子计算机断层扫描 磁共振成像 室管膜下结节 血管平滑肌脂肪瘤 心脏横纹肌瘤 室管膜下巨细胞星形细胞瘤 

分 类 号：R744.51[医药卫生—神经病学与精神病学] R445[医药卫生—临床医学]