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结节性硬化症复合体:11例影像学特征及文献复习

Tuberous sclerosis complex: Imaging characteristics in 11 cases and review of the literature.

作者信息

Hu Shan, Hu Dao-Yu, Zhu Wen-Zhen, Wang Liang, Wang Zi

机构信息

Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510080, China.

Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.

出版信息

J Huazhong Univ Sci Technolog Med Sci. 2016 Aug;36(4):601-606. doi: 10.1007/s11596-016-1632-5. Epub 2016 Jul 28.

Abstract

Tuberous sclerosis complex (TSC) is an uncommon multiorgan disorder that may present many and different manifestations on imaging. Radiology plays an important role in diagnosis and management, and can substantially improve the clinical outcome of TSC. Therefore, a comprehensive understanding of this disease is essential for the radiologist. The manifestations of TSC on computer tomography (CT) and magnetic resonance (MR) images were analyzed. Eleven patients with a clinical diagnosis of TSC were retrospectively reviewed. Central nervous system lesions included subependymal nodules (SENs) (11/11), subependymal giant cell astrocytomas (SEGAs) (2/11), cortical and subcortical tuber lesions (5/11), and white matter lesions (4/11). Of the 6 patients with abdominal scans, there were 6 cases of renal angiomyolipomas (AMLs), and one case of hepatic AMLs. Of the 4 patients undergoing chest CT, lung lymhangioleiomyomatosis (LAM) (2/4), and multiple small sclerotic bone lesions (2/4) were observed. Different modalities show different sensitivity to the lesion. Analysis of images should be integrated with patients' history in order to diagnose TSC.

摘要

结节性硬化症(TSC)是一种罕见的多器官疾病,在影像学上可能呈现多种不同表现。放射学在其诊断和管理中起着重要作用,并且可以显著改善TSC的临床预后。因此,放射科医生全面了解这种疾病至关重要。分析了TSC在计算机断层扫描(CT)和磁共振(MR)图像上的表现。对11例临床诊断为TSC的患者进行了回顾性研究。中枢神经系统病变包括室管膜下结节(SENs)(11/11)、室管膜下巨细胞星形细胞瘤(SEGAs)(2/11)、皮质和皮质下结节性病变(5/11)以及白质病变(4/11)。在6例进行腹部扫描的患者中,有6例肾血管平滑肌脂肪瘤(AMLs),1例肝AMLs。在4例接受胸部CT检查的患者中,观察到肺淋巴管平滑肌瘤病(LAM)(2/4)和多个小的硬化性骨病变(2/4)。不同的检查方式对病变显示出不同的敏感性。图像分析应与患者病史相结合以诊断TSC。

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