Diagnosis and management of pancreatic neuroendocrine tumor in von Hippel-Lindau disease

作     者：Kenji Tamura Isao Nishimori Tetsuhide Ito Ichiro Yamasaki Hisato Igarashi Taro Shuin 

作者机构：Department of UrologyKochi Medical SchoolNankokuKochi 783-8505Japan Nishimori's ClinicSakawaKochi 789-1233Japan Department of Medicine and Bioregulatory ScienceGraduate School of Medical SciencesKyushu UniversityHigashi-kuFukuoka 812-8582Japan 

出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)

年 卷 期：2010年第16卷第36期

页      面：4515-4518页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

基　　金：Supported by The Health and Labor Sciences Research Grant for a nationwide clinical survey and establishment of guidelines in the diagnosis and treatment for von Hippel-Lindau disease in Japan 

主　　题：Von Hippel-Lindau disease Pancreas Neuroendocrine tumor,Diagnosis Clinical protocols 

摘      要：The pancreatic manifestations seen in patients with von Hippel-Lindau(VHL) disease are subdivided into 2 categories:pancreatic neuroendocrine tumors(NET),and cystic lesions,including simple cyst and serous *** VHL-associated cystic lesions are generally asymptomatic and do not require any treatment,unless they are indistinguishable from other cystic tumor types with malignant *** pancreatic NET in VHL disease are non-functioning and have malignant potential,it is of clinical importance to find and diagnose these as early as *** will be recommended that comprehensive surveillance using dynamic computed tomography for abdominal manifestations,including pancreatic NET,should start from the age of 15 years in VHL *** sporadic non-functioning NET without VHL disease,in which surgical resection is generally recommended,VHL patients at lower metastatic risk of pancreatic NET should be spared the risks of operative resection.