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冯·希佩尔·林道病的影像学诊断病例报告及文献复习
引用本文:徐汇义,徐卓群,夏晓,陈宏伟,吴力源,李国良. 冯·希佩尔·林道病的影像学诊断病例报告及文献复习[J]. 中国医学影像技术, 2005, 21(1): 97-100
作者姓名:徐汇义  徐卓群  夏晓  陈宏伟  吴力源  李国良
作者单位:1. 无锡市第一人民医院泌尿外科,江苏,无锡,214002
2. 无锡市第一人民医院放射科,江苏,无锡,214002
摘    要:目的评价冯·希佩尔·林道病的影像学表现.方法二个家庭的四个病人接受了头部MR检查,腹部超声(US)和/或CT检查.结果四个病例呈现了冯·希佩尔·林道病的几乎所有主要临床征象,包括视网膜血管瘤、中枢神经系统血管母细胞瘤、内淋巴囊瘤、胰腺囊肿、肾上腺嗜铬细胞瘤、肾囊肿和肾透明细胞癌及附睾乳头状囊腺瘤等.结论影像学检查在冯·希佩尔·林道病的诊治中仍起着极其重要的作用.

关 键 词:冯·希佩尔·林道病  诊断显像
文章编号:1003-3289(2005)01-097-04
收稿时间:2004-11-10
修稿时间:2004-11-10

Imaging diagnosis of von Hippel Lindau disease:case report and review of literature
XU Hui-yi,XU Zhuo-qun,XIA Xiao,CHEN Hong-wei,WU Li-yuan and LI Guo-liang. Imaging diagnosis of von Hippel Lindau disease:case report and review of literature[J]. Chinese Journal of Medical Imaging Technology, 2005, 21(1): 97-100
Authors:XU Hui-yi  XU Zhuo-qun  XIA Xiao  CHEN Hong-wei  WU Li-yuan  LI Guo-liang
Affiliation:Department of Urology, Wuxi First People's Hospital, Jiangsu 214002, China;Department of Urology, Wuxi First People's Hospital, Jiangsu 214002, China;Department of Radiology, Wuxi First People's Hospital, Jiangsu 214002, China;Department of Radiology, Wuxi First People's Hospital, Jiangsu 214002, China;Department of Radiology, Wuxi First People's Hospital, Jiangsu 214002, China;Department of Radiology, Wuxi First People's Hospital, Jiangsu 214002, China
Abstract:Objective To evaluate the imaging manifestation of von Hippel Lindau (VHL) disease. Methods Four patients from two families with von Hippel Lindau disease underwent cranial magnetic resonance imaging (MRI), abdominal ultrasonography (US), and/or CT scans. Results The clinical and imaging manifestations of these cases demonstrated almost all of the major lesions with VHL disease, including retinal angiomas, hemangioblastomas (HBLs) of the central nervous system (CNS), endolymphatic sac tumors (ELSTs), pancreatic cysts, adrenal pheochromocytomas, renal cysts and renal clear cell carcinomas and epididymal cystadenomas. Conclusion Imaging techniques still play an important role in the evaluation and management of VHL disease.
Keywords:von Hippel-Lindau disease  Diagnostic imaging
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