结节性硬化症（TSC）：肾脏及肾外影像学表现

Tuberous Sclerosis Complex (TSC): Renal and Extrarenal Imaging.

作者信息

Gupta Shiva, Kang Hyunseon C, Faria Silvana C, Choyke Peter L, Kundra Vikas

机构信息

Department of Abdominal Imaging, Unit 1473, University of Texas MD Anderson Cancer Center; 1515 Holcombe Blvd, Houston, TX..

Department of Abdominal Imaging, Unit 1473, University of Texas MD Anderson Cancer Center; 1515 Holcombe Blvd, Houston, TX.

出版信息

Acad Radiol. 2022 Mar;29(3):439-449. doi: 10.1016/j.acra.2020.12.019. Epub 2021 Jan 22.

DOI:10.1016/j.acra.2020.12.019

PMID:33487538

Abstract

Tuberous sclerosis complex is a multiorgan syndrome manifesting with several benign and malignant tumors. Complications arising from renal abnormalities are a leading cause of death in patients with tuberous sclerosis complex. Renal cell carcinoma is relatively uncommon, occurring in 2%-4% of patients with tuberous sclerosis complex syndrome, but nonetheless can significantly contribute to morbidity and mortality. Extrarenal manifestations of tuberous sclerosis complex, including within the chest, abdomen and central nervous system, aid in diagnosis. Pathogenesis and management are also discussed, including the importance of the types of renal masses found in these patients.

摘要

结节性硬化症是一种多器官综合征，表现为多种良性和恶性肿瘤。肾脏异常引起的并发症是结节性硬化症患者死亡的主要原因。肾细胞癌相对少见，在结节性硬化症综合征患者中的发生率为2%-4%，但仍可显著影响发病率和死亡率。结节性硬化症的肾外表现，包括胸部、腹部和中枢神经系统的表现，有助于诊断。本文还讨论了其发病机制和治疗方法，包括这些患者中发现的肾肿块类型的重要性。