目的：探讨壶腹部临床病理特征及预后特点。方法：统计2017年1月至2019年12月间上海交通大学医学院附属瑞金医院行胰十二指肠切除手术标本中壶腹部腺癌的占比,分析患者的临床资料和病理学特征,根据2019年第五版《世界卫生组织消化系统肿瘤分类》中新的组织学分类标准将壶腹部腺癌重新分类,并统计各组织类型占比,分析随访及临床资料,总结预后不良的危险因素。结果：本组胰十二指肠切除标本中,6.95%（101/1 454）经术后病理证实为壶腹部腺癌。患者中位发病年龄为64岁。男女比例为46∶55。组织学类型占比分别为：肠型腺癌占19.80%（20/101）,胰胆管型腺癌占43.56%（44/101）,管状腺癌占36.63%（37/101）。101例患者中26例失访,余75例患者的随访时间为1~45个月,中位随访时间是18个月,术后1年生存率为78.58%,2年生存率为67.40%。Log-rank生存分析显示非肠型腺癌组（胰胆管型腺癌和管状腺癌）较肠型腺癌组总体生存率低（P=0.0415）;肿瘤细胞CK7阳性组较CK7阴性组总体生存率低（P=0.0425）。结论：壶腹部腺癌好发于老年人,无明显性别倾向;组织学类型以胰胆管型腺癌和管状腺癌为主,少数为肠型腺癌;非肠型腺癌、肿瘤细胞CK7阳性表达是患者预后不良的重要危险因素。

Objective: To evaluate the clinicopathologic features and prognosis of ampullary adenocarcinoma (AA). Methods: The proportion of AA in patients who underwent pancreaticoduodenectomy were calculated in Ruijin hospital from January 2017 to December 2019. The clinical data and pathological features were analyzed. According to the new histological classification standard in the fifth edition of the World Health Organization classification of digestive system tumors in 2019, AA was reclassified and the risk factors for poor prognosis were analyzed. Results: It revealed that AA account for 6.95%(101/1454) in patients undergone pancreaticoduodenectomies. The median age of onset was 64 years, and the male to female ratio was 46∶55. The proportion of histological sub-types were 19.80%(20/101) for intestinal-type adenocarcinoma, 43.56% (44/101) for pancreatobiliary-type adenocarcinoma and 36.63% (37/101) for tubular adenocarcinoma. Among these 101 patients, 26 patients were lost to follow-up, and the remaining 75 patients were followed up for 1-45 months, with a median postoperative follow-up period of 18 months. The 1-year survival rate of the patients was 78.58%, and the 2-year survival rate was 67.40%. Log-rank survival analysis showed difference in overall survival between the non-intestinal-type adenocarcinoma group (pancreaticobiliary-type adenocarcinoma and tubular adenocarcinoma) and the intestinal-type adenocarcinoma group (P=0.0415). There was also statistical difference in the overall survival between the CK7 positive group and the CK7 negative group of tumor cells (P=0.0425). Conclusions: AA occurres frequently in the elderly and there is no significant gender difference. Pancreatobiliary-type carcinoma and tubular adenocarcinoma are dominant histological types, and intestinal-type adenocarcinoma accounts for only a small portion. Non intestinal-type adenocarcinoma and CK7 positivity are risk factors for poor prognosis of AA.