Objective: To study the clinicopathologic features of intraductal papillary neoplasm of the bile duct(IPNB) and to analyze the diagnostic and therapeutic patterns. Methods: The data of 46 patients with IPNB undergoing surgery in Department of Hepatobiliary and Pancreatic Surgery, the Second Affiliated Hospital of Zhejiang University School of Medicine from January 2013 to November 2017 were retrospectively analyzed.There were 23 males and 23 females with age of (64±8)years.Patients were followed up by clinics and telephone inquiry.Categorical data were compared with χ(2) test or Fisher's exact test. Results: Abdominal pain(in 31 patients), fever (in 15 patients) and jaundice (in 11 patients) were the most common symptoms.Twenty-five patients were accompanied with cholangiolithiasis and 25 were accompanied with liver atrophy.Preoperative laboratory examination was mainly manifested as the abnormal liver function caused by biliary obstruction.Typical imaging findings included bile duct dilation (in 45 patients) and mass within bile duct (in 22 patients). All the patients were diagnosed as IPNB histopathologically.Among them, high-grade intraepithelial neoplasia and related adenocarcinoma were more common in mucus-hypersecretion IPNB ((13/15 vs. 51.6%(16/31))(χ(2)=5.331, P=0.021). Hepatectomy was performed in 25 patients, hepatectomy combined with biliary resection and reconstruction in 12 cases, biliary resection and reconstruction in 3 cases, pancreatoduodenectomy in 3 cases, hepatopancreaticoduodenectomy in 1 case, liver transplantation in 1 case and radiofrequency ablation in 1 case.Forty-one patients were followed up with a median of 30 (12, 41) months.Seven patients suffered recurrence and 6 died. Conclusion: IPNB is a rare disease with limited knowledge currently.Images are the main diagnositc means and surgery is the first choice.
目的： 探讨胆管导管内乳头状肿瘤(IPNB)的临床病理学特点及其诊治方法。 方法： 回顾性分析2013年1月至2017年11月于浙江大学医学院附属第二医院肝胆胰外科接受手术治疗的46例IPNB患者的临床病理学资料。男性23例，女性23例，年龄(64±8)岁。采用门诊复查结合电话询问的方式进行随访。计数资料的统计学分析采用χ(2)检验或Fisher精确检验。 结果： 腹痛(31例)、发热(15例)与黄疸(11例)是最常见的临床症状。25例患者伴胆管结石，25例患者伴肝脏萎缩。术前实验室检查主要表现为胆道梗阻所致肝功能异常，典型的影像学表现为胆管扩张(45例)，胆管内肿物(22例)。所有患者均经病理学检查证实为IPNB，其中黏液高分泌IPNB伴高级别上皮内瘤变或相关浸润性癌的比例(13/15)高于黏液低分泌IPNB(51.6%，16/31)(χ(2)＝5.331，P＝0.021)。25例患者接受肝切除术，12例接受肝切除术＋胆管切除重建术，3例接受胆管切除重建手术，3例接受胰十二指肠切除术，1例接受肝胰十二指肠切除术，1例接受同种异体肝移植术，1例接受肿瘤射频消融治疗。41例患者获得随访，中位随访时间为30个月[(12，41)个月]。随访期间，肿瘤复发7例，死亡6例。 结论： IPNB临床少见，目前临床上对其认识不足，影像学检查是其主要诊断手段，手术是目前首选的治疗方式。.
Keywords: Biliary tract neoplasms; Carcinoma, papillary; Diagnosis; Prognosis; Therapeutics.