摘要
多发性内分泌肿瘤Ⅰ型(MEN-1)由MEN-1抑癌基因突变所致,胰腺内分泌肿瘤是其主要病变之一,具有病理类型多样、发病早、多发等特点。基因测序是重要诊断方法,对基因突变携带者应定期筛查胰腺内分泌肿瘤。手术是控制激素过度分泌症状和预防转移的主要手段。
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.
出处
《中国实用外科杂志》
CSCD
北大核心
2010年第9期759-762,共4页
Chinese Journal of Practical Surgery