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投稿时间：2023-08-10 网络发布日期：2023-11-20
投稿时间：2023-08-10 网络发布日期：2023-11-20
中文摘要: 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。
Abstract: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.
keywords: Tuberous sclerosis complex Multiple organ tumors Computed tomography Magnetic resonance imaging Subependymal nodules Angiomyolipoma Cardiac rhabdomyoma Subependymal giant cell astrocytoma
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王雅菁， 卜顺林， 闵钢， 范小丽， 陈明亮.结节性硬化症12例临床影像分析[J].中国临床研究,2023,36(11):1708-1712,1717.
王雅菁， 卜顺林， 闵钢， 范小丽， 陈明亮.结节性硬化症12例临床影像分析[J].中国临床研究,2023,36(11):1708-1712,1717.