胆管内乳头状肿瘤的临床现状和研究进展

Clinical status and research progress of intraductal papillary neoplasm of the bile duct

胆管内乳头状肿瘤(IPNB)是1种罕见胆道肿瘤,以胆管扩张、胆管内乳头状增生或囊性改变,伴或不伴黏液分泌为特征。IPNB的病因及机制尚不清楚,已有的研究结果显示:胆管结石、华支睾吸虫感染等引起的胆汁淤积和胆道反复感染与IPNB的发生密切相关;Ras⁃MAPK、Wnt/β⁃catenin等信号通路在IPNB的进展过程中发挥重要作用。IPNB缺乏特异临床特征,其症状取决于肿瘤发生部位和胆管梗阻程度,主要表现为腹痛、梗阻性黄疸、发热和反复发作的胆管炎。根据上皮组织形态和免疫组织化学染色标志物,可将IPNB分为胰胆管型、肠型、胃型和嗜酸细胞型;根据主要病变所在位置,可将IPNB分为肝内型、肝外型和弥漫型,该分型具有较好临床指导作用。IPNB根治性手术治疗患者的预后优于姑息性治疗患者,建议所有符合适应证的患者行根治性治疗。笔者总结IPNB病因、进展机制、临床表现、病理学特征和诊断与治疗现状,并对其研究进展进行综述。

Intraductal papillary neoplasm of the bile duct(IPNB)is a rare type of biliary tract neoplasms characterized by biliary dilatation,papillary neoplasm or cystic changes,with or without mucous secretion.The etiology and mechanism of IPNB are still unclear.Studies have shown that its pathogenesis is closely related to cholestasis and repeated biliary tract infection caused by cholestasis and clonorchionchus infection and other diseases.Signaling pathways such as Ras‐MAPK and Wnt/β‐catenin play important roles in progression of IPNB.IPNB lacks specific clinical features.Its symptoms depend on the location of tumor and the degree of bile duct obstruction.The main manifestations are abdominal pain,obstructive jaundice,fever,and recurrent cholangitis.IPNB can be divided into pancreatobiliary type,intestinal type,gastric type and eosinophilic type according to epithelial tissue morphology and immunohistochemical staining markers.Based on the location of main lesions,IPNB can be divided into intrahepatic,extrahepatic and diffuse types.The prognosis of patients of IPNB receiving radical surgery is better than those receiving palliative treatment,and all patients with indications are recommended to receive radical surgery.The authors summarize the etiology,progression mechanism,clinical manifestations,pathological features,diagnosis and treatment status of IPNB,and provide a review of its research progress.