Abstract：
Intraductal papillary neoplasm of the bile duct (IPNB) is a heterogeneous disease similar to intraductal papillary mucinous neoplasm of the pancreas. These lesions have been recognized as one of the three major precancerous lesions in the biliary tract since 2010. In 2018, Japanese and Korean pathologists reached a consensus, classifying IPNBs into type l and type 2 IPNBs. IPNBs are more prevalent in male patients in East Asia and are closely related to diseases such as cholelithiasis and schistosomiasis. From a molecular genetic perspective, IPNBs exhibit early genetic variations, and different molecular pathways may be involved in the tumorigenesis of type 1 and type 2 IPNBs. The histological subtypes of IPNBs include gastric, intestinal, pancreaticobiliary, or oncocytic subtypes, but type 1 IPNBs typically exhibit more regular and well-organized histological features than type 2 IPNBs and are more commonly found in the intrahepatic bile ducts with abundant mucin. Due to the rarity of these lesions and the absence of specific clinical and laboratory features, imaging is crucial for the preoperative diagnosis of IPNB, with local bile duct dilation and growth along the bile ducts being the main imaging features. Surgical resection remains the optimal treatment for IPNBs, but negative bile duct margins and the removal of lymph nodes in the hepatic hilum significantly improve the postoperative survival rates for patients with IPNBs.
摘要：
胆管导管内乳头状肿瘤（IPNB）是一种异质性疾病，类似于胰腺导管内乳头状粘液性肿瘤。自2010年以来，这些病变已被认为是胆道三大癌前病变之一。2018年，日本和韩国病理学家达成共识，将IPNB分类为1型和2型IPNB。IPNBs多见于东亚男性患者，与胆石症、血吸虫病等疾病密切相关。从分子遗传学的角度来看，IPNBs表现出早期的遗传变异，1型和2型IPNBs的肿瘤发生可能涉及不同的分子途径。IPNBs的组织学亚型包括胃，肠,胰胆管,或嗜酸细胞亚型，但1型IPNBs通常表现出比2型IPNBs更有规律和组织良好的组织学特征,并且更常见于粘蛋白丰富的肝内胆管。由于这些病变的罕见性以及缺乏特定的临床和实验室特征，影像学对IPNB的术前诊断至关重要，以局部胆管扩张和沿胆管生长为主要影像学特征。手术切除仍然是IPNBs的最佳治疗方法，但是阴性的胆管边缘和肝门淋巴结的切除显着提高了IPNBs患者的术后生存率。
