十二指肠壶腹部神经内分泌癌的诊断与治疗（附七例报告）  

Diagnosis and management of neuroendocrine carcinoma of the ampulla of Vater ：A report of 7 cases

作　　者：陈胜[1] 吴卫泽[1] 王建承[1] 雷若庆[1] 费健[1] 韩天权[1] 彭承宏[1] 

机构地区：[1]上海交通大学附属瑞金医院普外科,200025

出　　处：《临床外科杂志》2010年第8期531-534,共4页Journal of Clinical Surgery

基　　金：上海市重点学科建设项目（编号：930204）

摘　　要：目的 探讨十二指肠壶腹部神经内分泌癌临床病理学特征、诊断和治疗方法.方法 回顾分析7例十二指肠壶腹部神经内分泌癌病例并结合文献,综合分析十二指肠壶腹部神经内分泌癌的临床病理学特征、诊断及治疗.结果 7例十二指肠壶腹部神经内分泌癌占同期十二指肠壶腹部肿瘤的4.32%（7/162）.主要临床症状为腹痛、梗阻性黄疸、消化道出血和贫血.术前影像学检查是诊断十二指肠壶腹部肿瘤的主要手段,其中1例患者在术前经内镜及内镜超声确诊.经组织病理及免疫组化诊断为大细胞神经内分泌癌3例、小细胞神经内分泌癌3例,不典型类癌1例.其中行胰十二指肠切除术5例,行十二指肠乳头部肿瘤局部切除术2例.所有患者均得到随访.全组中位生存期为23个月.结论 十二指肠壶腹部神经内分泌癌罕见.针对十二指肠壶腹部高级别神经内分泌癌进行根治性切除可能改善患者的预后.Objective This study was to determine the relative incidence, clinicopathological characteristics, diagnosis and management of neuroendocrine carcinoma of the ampulla of Vater at a single institution. Methods Sections from paraffin blocks of tumors of the ampulla of Vater taken from 162 patients who underwent pancreaticodudenectomy or local excision procedure between January 2003 and December 2008 were subjected to immunohistochemical analysis. The clinical and pathological data from 7 patients diagnosed with NEC of the ampulla of Vater were analyzed. Results The patients were 4 men and 3 women, ranging in age from 36 to 76 years （mean 58 years）. Operative procedures included pancreaticodudenectomy procedure in 5 patients and local excision in 2 patient. Histopathological examination revealed large- cell neuroendocrine carcinoma in 3 patients, small cell carcinoma in 3, and atypical carcinoid in 1. Four patients with high - grade neuroendocrine carcinoma who had undergone pancreaticodudenectomy procedure died at postoperatively 3,5,16 and 23 months. However,two patients with high -grade neuroendocrine carcinoma （one had undergone pancreaticodudenectomy procedure, one had undergone local excision） and one patient with atypical carcinoid who had undergone local excision showed no sign of recurrence and has been disease -free for more than 60,30,12 months respectively after surgery. The median survival time of all patients was 23 months. Conclusion Neuroendocrine carcinomas of the ampulla of Vater are rare. The high - grade neuroendocrine carcinomas behave aggressively and have a dismal prognosis despite aggressive surgical treatment.

关 键 词：神经内分泌癌 十二指肠壶腹部 

分 类 号：R735.31[医药卫生—肿瘤]