希佩尔-林道综合征中枢神经系统外病变的超声表现  

Sonographic Finding of Von Hippel-Lindau Syndrome Except the Pathological in Central Nervous System

作　　者：张敏[1] 覃伶伶[1] 符少清[1] 符小艳[1] 吴汤娜[1] 王丽[1] 

机构地区：[1]海南省人民医院超声科,海口市570311

出　　处：《中国超声医学杂志》2018年第1期89-92,共4页Chinese Journal of Ultrasound in Medicine

摘　　要：目的探讨希佩尔-林道综合征(VHL)的超声图像特点及诊断价值。方法回顾性分析9例VHL综合征患者中枢神经系统外病变的超声表现。结果双肾多发性囊肿6例,其中2例合并双肾透明细胞癌,肾单发囊肿1例;胰腺多发囊肿5例,其中1例合并胰岛细胞瘤,胰腺单发囊肿1例;肝脏多发血管瘤3例,单发血管瘤1例;肾上腺嗜铬细胞瘤1例;附睾囊腺瘤2例,肾透明细胞癌附睾转移1例。结论 VHL综合征累及多个器官,临床表现复杂多样,超声检查是发现及诊断中枢神经系统外病灶的首选方法,了解VHL综合征的超声表现,有利于疾病的早期诊断及治疗。Objective To assess values of sonographic in diagnosed of Von Hippel-Lindau syndrome（VHL）. Methods A Total of 9 patients with VHL were enrolled;the ultrasonic manifestations were retrospectively analyzed. Results Among 9 of them, 6 cases showed multiple renal cysts,2 of which combined both side cell clear cell carcino- ma,1 case was solitary renal cyst; 5 cases were multiple pancreatic cysts, 1 of which combined pancreatic islet cell tumor,1 case showed solitary pancreatic cyst~ 3 cases were multiple hepatic hemangioma and 1 case was solitary he- mangioma;1 case of adrenal pheochromocytoma~ 2 cases of cystadenoma of epididymis,1 case showed epididymal me- tastasis of clear cell renal carcinoma. Conclusions VHL syndrome involves multiple organs, and clinical manifesta- tions are complex and diverse, Ultrasonography is the preferred method for the diagnosis except the pathological in cen- tral nervous system. The ultrasonic manifestations of VHL syndrome is beneficial for the early diagnosis and treatment of the disease.

关 键 词：希佩尔-林道综合征 超声 

分 类 号：R445.1[医药卫生—影像医学与核医学] R596.1[医药卫生—诊断学]