VHL综合征的腹部超声影像学表现  

The Abdominal Sonographic Features of VHL

作　　者：邵玉红[1] 王彬[1] 张惠[1] 

机构地区：[1]北京大学第一医院超声科,北京市100034

出　　处：《中国超声医学杂志》2013年第5期470-474,共5页Chinese Journal of Ultrasound in Medicine

摘　　要：目的旨在探讨VHL综合征及其腹部超声影像学表现,客观评价超声在该病中的诊断价值。方法参照国际标准,回顾性分析的4例VHL病例的临床及腹部超声影像学资料。结果病例1:VHL-2B型。双肾多发透明细胞癌、囊肿,双肾上腺嗜络细胞瘤,胰腺多发无功能胰岛细胞瘤。病例2:VHL2C型。双肾上腺嗜铬细胞瘤,小脑血L管母细胞瘤。病例3:VHL-1型。双肾多发错构瘤、囊肿,胰腺多发囊肿,下丘脑血管母细胞瘤。病例4:VHL-1型。双肾多发肾透明细胞癌及囊肿、胰腺多发囊肿及囊腺瘤,有侧小脑及脊髓血管母细胞瘤。结论 VHL综合征是一组多发的、多器官的良、恶性肿瘤征候群,超声是发现VHL腹部肿瘤和VHL患者随访的首选检查。Objective To discuss the imaging characters and assess the value of the ultrasonography to diagnose the von Hippel-Lindau （VHL） disease. Methods Over a five-year period, this retrospective study enrolled VHL sub- jects which were in accordance with the VHL diagnostic criteria. The clinical and abdominal sonographic features of all cases were recorded. Results Four cases were confirmed as VHL. Case 1 was classified as BHL 2B, presenting with bilateral and multiple renal clear cell carcinomas and cysts, bilateral adrenal pheochromocytomas, and non-functional pancreatic islet tumors. Case 2 was type of VHL-2C, presenting with bilateral adrenal pheochromocytomas and right cerebellar medulloblastomas. Case 3 was type of VHL-1, presenting with multiple and bilateral renal angiomyolipo- mas, multiple renal cysts, multiple pancreatic cysts, and hypothalamic hemangioblastomas. Case 4 was type of VHL- 1, presenting with multiple and bilateral renal clear cell carcinomas and cysts, multiple pancreatic cysts and cystadeno- mas, right cerebellar and spinal hemangioblastomas. Conclusions VHL is a rare condition with multiple organs in- volved. Sonography is the preferred imaging tool of finding,comprehensive evaluation and following up this disease.

关 键 词：VHL超声腹部 

分 类 号：R445.1[医药卫生—影像医学与核医学] R73[医药卫生—诊断学]