关于遗传性胰腺内分泌肿瘤的一些思考  

Hereditary pancreas endocrine neoplasia

作　　者：陈曦[1] 李宏为[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院外科,上海200025

出　　处：《中国实用外科杂志》2010年第9期733-735,共3页Chinese Journal of Practical Surgery

摘　　要：胰腺内分泌肿瘤可发生于一系列遗传性疾病中,包括多发性内分泌肿瘤Ⅰ型、vonHippel-Lindau综合征、神经纤维瘤1型、结节性硬化病和Beckwith-Weideman综合征。遗传性胰腺内分泌肿瘤有独特的临床表现。近来在其基因发病机制方面有不少研究成果,但对其治疗方案仍有不少争议。Pancreatic endocrine neoplasia （PEN） can occur in as part of four inherited disorders including Multiple Endocrine Neoplasia type 1, von Hippel-Lindau disease, neurofibromatosis 1, tuberous sclerosis complex and Beckwith-Weideman syndrome. Over the last few years there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization, medical and surgical treatment of the PENs in these patients. These hereditary PENs present a number of unique clinical manifestation and management issues. In this article these areas are briefly reviewed as well as the current state of knowledge of the PENs in these disorders and the controversies that exist in their management are briefly summarized and discussed.

关 键 词：胰腺内分泌肿瘤 神经内分泌肿瘤 多发性内分泌肿瘤1型 vonHippel-Lindau综合征 神经纤维瘤1型 结节性硬化病 Beckwith-Weideman综合征 基因 

分 类 号：R6[医药卫生—外科学]