von Hippel—Lindau病的影像表现  

Imaging appearances of von Hippel-Lindau disease

作　　者：马小龙[1] 魏伟[2] 汪建华[1] 陆建平[1] 王莉[1] 刘崎[1] 蒋慧[3] 

机构地区：[1]第二军医大学附属长海医院影像医学科,上海200433 [2]第二军医大学遗传研究所 [3]第二军医大学附属长海医院病理科,上海200433

出　　处：《中华放射学杂志》2013年第4期344-348,共5页Chinese Journal of Radiology

基　　金：长海医院“1255计划”学科特色提升项目（CH125520804）

摘　　要：目的探讨yon Hippel—Lindau病的影像表现。方法回顾性分析10例符合von Hippel—Lindau病基因诊断标准患者的临床和影像资料。7例行cT腹部平扫加动态增强检查，3例行MRI腹部平扫加增强检查。10例均行颅脑MRI平扫加增强检查。对其影像表现和累及的脏器进行分析。结果所有患者均有胰腺多发囊肿。8例有肾脏多发囊肿。3例伴有胰腺多发胰岛细胞瘤，其中1例伴有胰腺浆液性囊腺瘤，1例伴有左侧肾上腺嗜铬细胞瘤。3例有双侧肾癌（其中1例右·肾已切除），1例患右侧肾癌。2例有小脑血管母细胞瘤，其中1例伴有脊髓多发血管母细胞瘤和左眼血管母细胞瘤。2例除胰腺多发囊肿和肾囊肿外未发现肿瘤。结论胰腺多发囊肿合并肾脏多发囊肿对von Hippel—Lindau病具有高度提示意义。胰腺多发内分泌瘤、胰腺浆液性囊腺瘤、肾上腺嗜铬细胞瘤、肾癌、中枢神经系统多发血管母细胞瘤和视网膜血管母细胞瘤也可伴生于von Hippel-Lindau病。Objective To assess imaging appearances of von Hippel-Lindau disease （VHLD）. Methods The clinical and imaging data of ten patients who met VHLD gene diagnostic criteria were retrospectively analyzed. Seven underwent CT abdominal scan, three underwent MRI abdominal scan. All patients underwent MRI cranial scan. Eight patients were treated by surgery, and three of them underwent endoscopic ultrasound guided aspiration biopsy of pancreatic tumors. Results All patients had multiple cysts of the pancreas, eight patients suffered multiple cysts of kidneys. Three patients suffered multiple islet cell tumors of pancreas ： one was accompanied with pancreatic serous cystadenoma, another was accompanied with left adrenal pheochromocytoma. Three patients had bilateral renal cell carcinoma （ one of them had the right kidney cut off）, one patient had right renal cell carcinoma. Two patients suffered cerebellar hemangioblastomas, and one of them was accompanied with mutiple spinal hemangioblastomas and the left retinal hemangioblastoma. Two patients with multiple cysts of pancreas and kidneys had not been found any tumor. Conclusion Multiple cysts of pancreas and kidney are highly suggestive of VHLD. At the same time, endocrine tumors of pancreas, serous cystadenoma of pancreas, adrenal pheochromocytoma, renal carcinomas, multiple hemangioblastomas of central nervous system and retinal hemangioblastoma can also occur in VHLD.

关 键 词：胰腺肿瘤 血管母细胞瘤 肾肿瘤 

分 类 号：R596[医药卫生—内科学]