摘要目的:分析Vaterian系统(胆总管和Vater壶腹)腺肌瘤样增生(AH)的特征，为该病的诊治提供思路。方法:回顾性分析解放军总医院第一医学中心2005年1月至2021年12月经术后病理诊断为Vaterian系统AH 17例患者资料，其中男性12例，女性5例，年龄(58.4±11.3)岁。分析患者临床表现、治疗和术后病理情况等。显微镜下AH周围非癌变区域管壁黏膜上皮异型增生患者纳入AH伴异型增生组( n＝8)，无异型增生纳入对照组( n＝9)。比较两组临床特征。 结果:主要临床症状为腹痛8例，其次是黄疸7例、发热2例。术前影像学检查显示占位性病变10例，6例未见明显病变亦无结石或胆道损伤狭窄等而存在异常增粗的肝内外胆管。16例行根治性胰十二指肠切除术。1例因胆道结石合并胆道梗阻行肝外胆道切除联合胆肠吻合术。有3例合并恶性肿瘤，其中1例为Vater壶腹AH自身发生癌变，另外2例肿瘤位于AH周围的管壁黏膜上皮，分别为胆管癌、壶腹癌。AH伴异型增生组和对照组AH患者年龄、性别、胆管结石、胆管炎、合并癌变以及肝功能指标等比较，差异均无统计学意义(均 P>0.05)。 结论:Vaterian系统AH临床表现或影像学检查无明显特征，难以与恶性肿瘤鉴别，该类患者建议行手术治疗。

abstractsObjective:To study the features of adenomyomatous hyperplasia (AH) of the Vaterian system (common bile duct and ampulla of Vater) to help in the diagnosis and management of this disease.Methods:A retrospective analysis on the data of 17 patients who had a postoperative pathological diagnosis of AH of the Vaterian system treated from January 2005 to December 2021 at the First Medical Center of the PLA General Hospital was carried out with 12 males and 5 females, aged (58.4±11.3) years. The clinical presentations, treatment and postoperative pathology of these patients were analyzed. Patients with dysplasia of the tubular mucosal epithelium in the non-cancerous area around the AH under microscopy were included in the AH with dysplasia group ( n=8), and those without dysplasia were included in the control group ( n=9). The clinical characteristics of the two groups were compared. Results:The main clinical symptoms were abdominal pain in 8 patients, jaundice in 7 patients and fever in 2 patients. Preoperative imaging showed 10 cases of occupying lesions and 6 cases of abnormally dilated intrahepatic and extrahepatic bile ducts without obvious lesions or stones or biliary tract injury stenosis. Sixteen patients underwent radical pancreaticoduodenectomy, and 1 patient underwent extrahepatic biliary resection combined with choledochojejunostomy for bile duct obstruction due to biliary stones, 3 patients had combined malignant tumors, 1 patient had a carcinoma of AH origin at the ampulla of Vater, and the other 2 patients had neoplastic lesions in the mucosal epithelium adjacent to the AH (cholangiocarcinoma and ampullary carcinoma, respectively). There were no significant differences in age, gender, bile duct stones, cholangitis, combined carcinoma and liver function indexes between the two groups of patients with AH of the Vaterian system (all P>0.05). Conclusion:Adenomyomatous hyperplasia of the Vaterian system was difficult to distinguish preoperatively from malignant tumors basing on its clinical presentations or imaging findings. Such patients are recommended to be treated surgically.