摘要胆管内乳头状肿瘤(IPNB)是一类罕见的胆道肿瘤,以胆管内乳头状生长、胆管扩张、大量黏液分泌为特征.2010年WHO分类中,将其分为胆管内乳头状癌及癌前病变.病因和发病机制目前尚不清楚,可能与胆管结石及华支睾吸虫病有关.IPNB的临床表现以腹部疼痛、急性胆管炎及梗阻性黄疸最为常见.实验室检查无特异性,通常显示梗阻性黄疸所致的肝功能异常.常见的影像学表现为胆管扩张及管腔内肿物.CT、MRI及内镜检查通常被用于评估肿瘤的部位及范围.根据肿瘤上皮细胞的形态,IPNB可分为胰胆管型、肠型、胃型及嗜酸性细胞型4种表型.手术切除是IPNB的主要治疗方法,手术方式与肝内胆管细胞癌及肝外胆道肿瘤大体相同,具体手术方式根据肿瘤部位及范围而定.虽然目前有许多研究试图阐明IPNB病变发展过程中的分子基因改变,但具体机制仍不清楚,仍需进一步研究.

abstractsIntraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct tumors characterized by papillary growth within the dilated bile duct lumen and secretion with a large amount of mucins.IPNB is an intraductal papillary cancer of the bile duct or a preinvasive lesion classified by the 2010 World Health Organization (WHO) classification.Although the specific etiology and pathogenesis are unclear,IPNB is known to two major risk factors:hepatoliathiasis and clonorchiasis.The clinical manifestations of IPNB include intermittent abdominal pain,acute cholangitis and obstructive jaundice.Results of laboratory test show abnormal liver function resulting from obstruction of bile duct,but it's not specific.The most common imaging findings for IPNB are bile duct dilatation and intraductal masses.Computed tomography,magnetic resonance image and cholangiography are usually performed to access tumor location and extension.According to morphology of the tumor epithelial cells,IPNB are classified into the pancreticbiliary,intestinal,gastric and oncocytic types.The surgical resection is a major treatment of IPNB.In principle,IPNB should be resected in a manner similar to that employed for other types of intrahepatic cholangiocarcinomas and extrabepatic bile duct carcinomas.The type of surgical procedure for IPNB depends upon tumor location and extension.Although several investigations have been conducted for illuminating molecular genetic changes during the development and progression of IPNB,the specific mechanism is still not clear,and further study is needed.