胆管导管内乳头状黏液性肿瘤的临床病理特征和预后

Clinical characteristics and prognosis of intraductal papillary mucinous neoplasm of the biliary tract

目的探讨胆管导管内乳头状黏液性肿瘤(IPMN-B)的临床病理特征、外科诊疗方式和预后。方法回顾性分析2014年1月至2018年1月于西安市第三医院和吉林大学第一医院就诊的IPMN-B患者的临床病理资料,生存分析采用Kaplan-Meier法。16例患者中,男10例,女6例,中位年龄57岁。结果临床表现以黄疸(11例)、上腹部疼痛(12例)和高热寒战(4例)为主;合并胆管结石14例,合并肝脏萎缩2例;肿瘤大小为(2.6±0.7)cm。16例患者病理诊断均为IPMN-B,癌变11例,其中浸润癌5例(神经浸润4例),无脉管浸润;无浸润6例。16例患者病理类型中,胰胆管型8例,胃型5例,肠型2例,嗜酸细胞型1例。实验室检查可见肝功异常12例,胆红素升高9例,糖类抗原199升高8例,癌胚抗原升高4例;影像学检查可见胆管内肿物9例,胆管扩张14例。全组患者均行手术切除,肝左外叶切除1例,左半肝切除1例,胆管肿物切除+胆肠吻合12例,胰十二指肠切除2例,均可达到根治性切除。15例患者获得有效随访,死亡4例。患者术后中位复发时间为31个月(95%CI为23~39个月),1、2、3年复发率分别为6.7%、40.0%和73.3%,中位总生存时间为35个月(95%CI为23~47个月),1、2、3年生存率分别为100%、80.0%和53.3%。结论IPMN-B是一种罕见的胆道系统肿瘤,早期诊断困难,以手术治疗为主,预后较好。

Objective To study the clinical features,diagnosis,surgery treatment and prognosis of intraductal papillary mucinous neoplasm of the biliary tract(IPMN-B).Methods The data of 16 patients with IPMN-B admitted to The First Hospital of Jilin University and Xi′an Third Hospital from January 2014 to January 2018 were retrospectively analyzed.Kaplan-Meier method was used to conduct the survival analysis.These patients included 10 males and 6 females,the median age was 57 years.Results Clinical manifestations were mainly jaundice(11 cases),upper abdominal pain(12 cases)and hyperpyretic chills(4 cases),combined with bile duct stones(14 cases)and hepatic lobe atrophy(2 cases).The average size of the tumor was(2.6±0.7)cm.All of 16 cases were diagnosed as IPMN-B,including 5 cases of invasive carcinoma(4 cases were perineural invasion)without vascular invasion and 6 cases of non-invasion carcinoma.The pathological type included 8 cases of pancreaticobiliary duct type,5 cases of gastric type,2 cases of intestinal type and 1 case of eosinophils type.Laboratory tests showed abnormal liver function(12 cases),increased direct bilirubin(9 cases),increased carbohydrate antigen199(CA199,8 cases)and carcinoembryonic antigen(CEA,4 cases).The enhanced CT detection showed 9 cases of intrabile duct mass and 14 cases of bile duct dilatation.Surgical resection is the main treatment method,including 2 cases of partial resection of liver,12 cases of bile duct mass resection combined with choledochojejunostomy and 2 cases of pancreatoduodenectomy,all of whom achieved R0 resection.Fifteen patients were followed up and 4 died during the period.The median postoperative progression free survival was 31 months(95%CI:33-47 months),and the recurrence rate at 1 year,2-years and 3-years were 6.7%,40.0%and 73.3%,respectively.The median overall survival was 35 months(95%CI:23-47 months),and the 1 year,2-years,and 3-years cumulative survival rates were 100%,80.0%,and 53.3%,respectively.Conclusions IPMN-B is a rare tumor of biliary tract system,which is difficult to be diagnosed early.The main treatment is surgical resection,which can achieve a good prognosis.