von Hippel‑Lindau综合征合并胰腺病变的临床影像学特征及预后分析  

Clinical imaging features and prognosis of von Hippel‑Lindau syndrome associated with pancreatic lesions

作　　者：陈秋争 周靖程 刘宗昊 郭小超[3] 刘伟康 田孝东[1] 龚侃[2] 杨尹默[1] Chen Qiuzheng;Zhou Jingcheng;Liu Zonghao;Guo Xiaochao;Liu Weikang;Tian Xiaodong;Gong Kan;Yang Yinmo(Department of General Surgery,Peking University First Hospital,Beijing 100034,China;Department of Urology Surgery,Peking University First Hospital,Beijing 100034,China;Department of Radiology,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院普通外科,北京100034 [2]北京大学第一医院泌尿外科,北京100034 [3]北京大学第一医院影像科,北京100034

出　　处：《中华消化外科杂志》2023年第5期650-656,共7页Chinese Journal of Digestive Surgery

基　　金：国家自然科学基金(81871954、82171722、82271764、82141103)。

摘　　要：目的探讨von Hippel‑Lindau(VHL)综合征合并胰腺病变的临床影像学特征和预后。方法采用回顾性病例对照研究方法。收集2010年9月至2022年8月北京大学第一医院收治161例VHL综合征患者的临床病理资料;男83例,女78例;发病年龄为27.0(8.0~66.0)岁。观察指标:(1)VHL综合征合并胰腺病变患者影像学检查结果。(2)VHL综合征合并胰腺病变患者的临床特征。(3)VHL综合征合并胰腺囊性病变患者的临床病理因素比较。(4)VHL综合征合并胰腺神经内分泌肿瘤(pNENs)患者的临床病理因素比较。(5)VHL综合征合并胰腺病变患者的治疗及预后。正态分布的计量资料以x±s表示,组间比较采用独立样本t检验;偏态分布的计量资料以M(范围)表示,组间比较采用非参数检验。计数资料以绝对数表示,组间比较采用χ^(2)检验。结果(1)VHL综合征合并胰腺病变患者影像学检查结果:161例VHL综合征患者中,合并胰腺病变151例,未发现胰腺病变10例。151例VHL综合征合并胰腺病变患者中,合并胰腺囊性病变136例,合并pNENs 34例,同时合并胰腺囊性病变和pNENs 22例,无法准确判断胰腺病变类型3例。(2)VHL综合征合并胰腺病变患者的临床特征:151例VHL综合征合并胰腺病变患者发病年龄为33.0(14.0~68.0)岁;其中外显子1、2、3、其他类型基因位点突变分别为51、16、43、41例;VHL 1型116例,VHL 2型35例;VHL综合征家族史92例,无VHL综合征家族史59例;合并肾细胞癌127例,合并中枢神经系统病变112例,合并视网膜血管母细胞瘤46例(部分患者合并多种病变)。(3)VHL综合征合并胰腺囊性病变患者的临床病理因素比较:136例VHL综合征合并胰腺囊性病变患者发病年龄,VHL综合征分型(VHL 1型、VHL 2型),合并肾细胞癌分别为32.5(14.0~68.0)岁,110、26例,115例;25例VHL综合征未合并胰腺囊性病变患者上述指标分别为22.0(8.0~64.0)岁,13、12例,14例,两者上述指标比较,差异均有统�Objective To investigate the clinical imaging features and prognosis of von Hippel‐Lindau(VHL)syndrome associated with pancreatic lesions.Method The retrospective case‐control study was conducted.The clinicopathological data of 161 patients with VHL syndrome who were admitted to Peking University First Hospital from September 2010 to August 2022 were collected.There were 83 males and 78 females,with age of onset as 27.0(range,8.0−66.0)years.Observation indicators:(1)imaging results of VHL syndrome associated with pancreatic lesions;(2)clinical characteristics of VHL syndrome associated with pancreatic lesions;(3)comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic cystic lesions;(4)comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic neuroendocrine neoplasms(pNENs).(5)Treatment and prognosis of patients with VHL syndrome associated with pancreatic lesions.Measurement data with normal distribution were represented as Mean±SD,and comparison between groups was conducted using the independent sample t test.Measurement data with skewed distribution were represented as M(range),and comparison between groups was conducted using the non‐parameter test.Count data were described as absolute numbers,and comparison between groups was conducted using the chi‐square test.Results(1)Imaging results of VHL syndrome associated with pancreatic lesions.Of the 161 patients with VHL syndrome,there were 151 patients associated with pancreatic lesions and 10 patients not associated with pancreatic lesions.Of the 151 patients with VHL syndrome associated with pancreatic lesions,there were 136 patient with pancreatic cystic lesions and 34 patients with pNENs,22 patients with both pNENs and pancreatic cystic lesions,and the type of pancreatic lesions could not be accurately determined in 3 cases.(2)Clinical characteristics of VHL syndrome associated with pancreatic lesions.The age of onset in 151 patients with VHL syndrome associated with

关 键 词：冯·希佩尔‑林道病 胰腺病变 胰腺神经内分泌肿瘤 胰腺囊性病变 诊断 

分 类 号：R735.9[医药卫生—肿瘤]