以胰腺多发囊肿为主要症状的VHL病一例报告并文献复习  

One case report on VHL disease with multiple cysts of the pancreas as the main symptom

作　　者：李友伟[1] 谢辉[1] 麦刚[1] 刘山[1] 马春[1] 刘凤芬 谭洁[1] Li Youwei;Xie Hui;Mai Gang;Liu Shan;Ma Chun;Liu Fengfen;Tan Jie(Department of Hepatopancreatobiliary Surgery, People’s Hospital of Deyang City, Deyang 618000, China)

机构地区：[1]德阳市人民医院,618000

出　　处：《中华内分泌外科杂志》2019年第3期259-262,共4页Chinese Journal of Endocrine Surgery

基　　金：国家自然科学基金(31771580).

摘　　要：Von Hippel-Lindau(VHL)病是一组涉及多系统、多器官的良恶性肿瘤症候群疾病,我们近期收治了一例以胰腺多发囊性占位为初始临床症状的病例,经多学科协作,发现小脑、脊髓、视网膜均存在无临床症状的血管母细胞瘤,从而诊断Von Hippel-Lindau病。既往的文献病例报道中,单病例较少存在如此众多部位的中枢神经系统病变,且影像学表现典型。本文旨在结合文献复习,提高以胰腺病变为主要始发症状的VHL病的诊治能力。Von Hippel-Lindau (VHL) disease is a benign or malignant tumor syndrome which involves multiple systems and organs. Recently, a patient was diagnosed and hospitalized with an initial clinical symptom of pancreatic multiple cystic space-occupying. Multidisciplinary examination and consultation confirmed that there were hemangioblastomas in cerebella, spinal cord and retina, but with no clinical symptoms;therefore, it was diag-nosed as VHL disease. According to previous case reports, only a few single cases revealed lesions in so many parts of the central nervous system with typical imaging manifestations. We present an overview and aim to improve the diagnosis of VHL disease with the initial clinical symptom of pancreatic lesions.

关 键 词：VHL病 血管母细胞瘤 胰腺囊肿 

分 类 号：R576[医药卫生—消化系统]