Abstract: Intraductal papillary neoplasm of the bile duct （IPNB） is a rare type of tumor, characterized by dilated bile ducts filled with a papillary or villous biliary neoplasms and secretion with a large amount of mucins in some cases. It is difficult to obtain a positive and correct diagnosis before surgery and the most common presenting symptoms are abdominal pain, jaundice and cholangitis. It is likely to make a definite diagnosis based on the accumulated knowledge of the specific clinical features of IPNB. Currently the definition of IPNB remains controversial and the differential diagnosis of papillary cholangiocarcinoma and IPNB remain challenging. Other pathological features include multifocality and different proportion of invasive components. Patients with pancreaticobiliary subtype have worse prognosis and mucin core protein （MUC）-1 is the most important immunohistochemical marker related to the survival. IPNB have a variant of distinctive imaging characteristics and it is possible to determine whether an associated invasive carcinoma exist before surgery according to CT combined with MRCP imaging finding. Surgery is the primary treatment and the overall postoperative survival is better than general cholangiocarcinoma.

Key words: bile tract neoplasms, papillary neoplasms, diagnosis, mucin core proteins

摘要： 胆管内乳头状肿瘤（IPNB）是一类少见的胆道肿瘤,以肿瘤细胞在胆管内呈乳头状或绒毛状生长、胆管扩张,部分合并黏液分泌为特征。IPNB术前诊断困难,病史以腹痛、黄疸、胆管炎常见,如能够对其临床特点有深入的认识,有可能提高术前的诊断率。IPNB目前仍在病理学定义上仍存在争议,乳头状胆管癌是否归属于IPNB仍无定论。病理上可合并多发且性质不同的病变,应注意浸性型癌的比例。病理学分类上胰胆管亚型提示预后较差,黏液核心蛋白（mucin core protein）-1是最重要的免疫组化指标。IPNB影像学检查有其固有的特点,通过联合CT、MRCP检查有可能在术前判断是否存在浸润性癌,从而对手术方式及预后有一定的指导作用。手术切除是目前的首选治疗方式,IPNB患者术后的总体预后较胆管癌好。

关键词: 胆道肿瘤, 乳头状肿瘤, 诊断, 黏蛋白