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, von Hippel-Lindau综合征, 神经纤维瘤1型, 结节性硬化病, Beckwith-Weideman综合征, 基因

Abstract:

Hereditary pancreas endocrine neoplasia        CHEN Xi, LI Hong-wei. Department of Surgery, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai200025, China
Corresponding author: LI Hong-wei, E-mail: hwl@263.net
Abstract    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.

Key words: pancreas endocrine neoplasis, neuroendocrine tumors, multiple endocrine neoplasia type 1, von Hippel-Lindau disease, neurofibromatosis 1, tuberous sclerosis, Beckwith-Weideman syndrome, gene