Von Hippel-Lindau综合征腹部多脏器病变的影像诊断分析

Imaging diagnosis of multiple organ diseases in Von Hippel-Lindau syndrome

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.