Von Hippel-Lindau综合征累及肾脏、胰腺及肾上腺的CT表现  

Renal、pancreatic and adrenal involvement in Von Hippel-Lindau syndrome: CT findings

作　　者：陈美容[1] 蔡华崧[2] 董帜[2] 罗宴吉[2] 冯仕庭[2] 

机构地区：[1]广东省江门市中心医院放射科,529071 [2]中山大学附属第一医院放射科,广州510080

出　　处：《中华腔镜泌尿外科杂志（电子版）》2013年第4期23-26,共4页Chinese Journal of Endourology(Electronic Edition)

摘　　要：目的总结Von Hippel-Lindau syndrome(VHL综合征)累及肾脏、胰腺及肾上腺的CT表现,以提高对本病的认识。方法收集临床符合VHL综合征诊断标准且有腹部受累的病例9例,回顾性分析其临床、病理资料及多层螺旋CT(MSCT)表现。9例全部行MSCT平扫及增强检查。结果9例均有双肾多发病变,分为3种类型:单纯性囊肿、囊实性肿块和实性肿块,其中6例3种类型病灶混合存在,3例仅有双肾多发单纯性囊肿。3例行肾肿块切除,术后病理为透明细胞癌。7例胰腺可见多发囊肿,其中4例囊壁见钙化,1例合并胰岛细胞瘤。3例肾上腺可见嗜铬细胞瘤,2例为双侧。5例具有家族遗传史。结论 VHL综合征可累及腹部多个器官,表现复杂多样,熟悉掌握其MSCT表现有利于疾病的早期、全面诊断。Objective To summarize the CT findings and to enhance the understanding of renal、 pancreatic and adrenal involvement in Von Hippel-Lindau syndrome.Methods Collect 9 cases of patients who were clinically consistent with VHL syndrome diagnostic criteria and abdominal involvement,and the clinical,pathological data and multi slice spiral CT（MSCT） performance were analyzed retrospeclively.All underwent MSCT scan and enhance the inspection.Results Multiple bilateral renal lesions were detected in 9 patients.The renal lesions were classified as cystic,cystic with solid components and solid.Multiple combined renal lesions were found in 6 patients,and multiple simple cysts in 3 patients.Renal tumor resection was performed in 3 patients,and the renal masses were diagnosed as clear cell carcinoma by pathology.Pancreatic cysts were found in 7 patients,calcifications were detected in 4 patients,and pancreatic islet cell tumor was detected in 1 patient.Adrenal pheochromocytomas were found in 3 patients,including 2 bilateral cases.Five patients have family history.Conclusions Multiple abdominal organs can be involved in VHL syndrome,familiarity with the CT imaging features of VHL will be helpful to the early and comprehensive diagnosis.

关 键 词：Von Hippel-Lindau综合征 体层摄影术 X线计算机 

分 类 号：R816.5[医药卫生—放射医学]