摘要
目的分析Von Hippel-Lindau (VHL)综合征相关腹部脏器病变的影像特点,以提高对该病的全面诊断。方法回顾性分析27例VHL综合征患者的腹部影像特征。17例行腹部CT平扫加增强检查,3例行腹部MRI平扫加增强检查,7例同时行腹部CT和MR平扫加增强检查。结果 27例中6例有家族史。24例胰腺病变,包括2例多囊胰并胰腺神经内分泌肿瘤(其中1例肝转移),22例多囊胰。14例肾肿瘤,包括1例双侧肾癌并错构瘤,9例双侧肾癌,1例单侧肾癌,2例双侧错构瘤,1例单侧错构瘤。5例肾上腺肿瘤,包括3例双侧肾上腺嗜铬细胞瘤,2例单侧肾上腺嗜铬细胞瘤。2例胆总管神经内分泌肿瘤。2例附睾肿瘤,双侧内胚窦瘤与囊腺瘤各1例。本组中最易受累脏器部位为胰腺、肾脏及肾上腺。同时累及两个及两个以上脏器者15例(55%)。同时累及两个器官者,以胰腺与肾脏同时受累最常见,共8例(30%),其中多囊胰与肾癌共6例,多囊胰与错构瘤1例,多囊胰、肾癌与错构瘤1例。其他累及两个脏器的包括:累及胰腺与胆总管1例、胰腺与肾上腺各1例。同时累及三个器官者5例(19%),均累及胰腺与肾脏,2例累及肾上腺(均为嗜铬细胞瘤)、2例累及附睾(内胚窦瘤与囊腺瘤各1例)、1例累及胆总管(神经内分泌肿瘤)。结论 VHL综合征常表现为腹部多脏器受累,其中以多囊胰与肾肿瘤为最常见表现,此外需注意观察肾上腺、胆总管、附睾。
Objective To investigate image feature of Von Hippel-Lindau(VHL)syndrome involved abdominal organs.Methods Abdominal CT imaging features of 27 patients with VHL syndrome were retrospectively analyzed.17 cases underwent abdominal unenhanced and contrast-enhanced CT.3 cases underwent abdominal unenhanced and contrast-enhanced MRI.7 cases underwent abdominal unenhanced and contrast-enhanced CT and MR.Results Six of the 27 patients had family history.There were 24 cases of pancreatic lesions,including 2 cases of polycystic pancreatic pancreatic neuroendocrine tumors(1 case of liver metastasis),22 cases of polycystic pancreas.There were 14 cases of renal tumors,including 1 case of bilateral renal cell carcinoma with hamartoma,9 cases of bilateral renal cell carcinomas,1 case of unilateral renal cell carcinoma,2 cases of bilateral hamartomas,and 1 case of unilateral hamartoma.5 cases of adrenal tumors,3 cases of bilateral adrenal pheochromocytoma,2 cases of unilateral adrenal pheochromocytoma.There were 2 cases of common bile duct neuroendocrine tumors and 2 cases of epididymal tumor,bilateral yolk sac tumors and cystal adenoma in 1 case,respectively.The most susceptible organs in this group were the pancreas,kidneys and adrenal glands.Two or more organs were involved at the same time in 15 cases(55%).The most common type was involvement of both pancreas and kidneys(8 cases,30%),including 6 cases of polycystic pancreas and renal carcinoma,1 case of polycystic pancreas and hamartoma,polycystic pancreas,1 case of renal carcinoma and hamartoma.And there were 1 case involving pancreas and common bile duct and 1 case involving pancreas and adrenal gland carcinoma.Three abdominal organs were involved in 5 case(19%).Pancreas and kidneys were involved,and adrenal gland(n=2),epididymis(n=2),and common bile duct(n=1)could also be harmed.Conclusion VHL syndrome often manifests as multiple organs involvement in the abdomen.Polycystic pancreas and renal tumors are common.Adrenal gland,common bile duct and epididymis should be focused.
作者
何铭
张繁
张皓钦
肖煦阳
孙灿辉
王焕军
HE Ming;ZHANG Fan;ZHANG Hao-qin;XIAO Xu-yang;SUN Can-hui;WANG Huan-jun(Department of Radiology,Hospital of Traditional Medicine of Liuzhou,Guangxi 545001,China)
出处
《影像诊断与介入放射学》
2019年第1期60-65,共6页
Diagnostic Imaging & Interventional Radiology
基金
广东省自然科学基金(编号:2017A030313902)
