多发性内分泌肿瘤Ⅰ型相关的胰腺内分泌肿瘤

Multiple endocrine neoplasia type 1 related pancreatic endocrine tumors

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.