关键词: 多发性内分泌肿瘤Ⅰ型, 胰腺内分泌肿瘤, 基因

Abstract:

Multiple endocrine neoplasia type 1 related pancreatic endocrine tumors        CHEN Xi. Department of General Surgery, Ruijin Hospital,Shanghai Jiaotong University School of Medicine, Shanghai200025,China
Abstract    The endocrine pancreas-duodenum is often be involved in multiple endocrine neoplasia type 1 (MEN-1) which is an autosomal-dominant syndrome as a result of inactivating mutations of the MEN-1 tumor suppressor gene on chromosome 11q13 encoding for menin. The MEN-1 related pancreas-duodenum endocrinopathies are multicentric with early onset and lead to controversial strategy in management.  Gene consequence helps diagnosis. Surveillance and screening for pancreas-duodenum endocrine tumors are recommended in presymptomatic gene carriers. An aggressive surgical approach is intended to control the functional syndromes and malignant potential for nodal or distant metastasis.

Key words: multiple endocrine type 1, pancreas endocrine tumor, gene