Studies establishment of high rink group for bile duct cancer

胰胆管畸形（PBM）是一种位于十二指肠壁外的胰腺和胆管结合的先天性异常，是最近发现的一种疾病实体，常与先天性胆总管囊肿（CCBD）相关，其胚胎病因被认为与先天性胆总管囊肿不同，且临床特征多样，如PBM和CCBD患者可出现消化系统疾病。通过对250例PBM患者（169例为良性肝胆胰疾病，81例为恶性疾病）的分析，回顾了PBM患者的病理生理和临床方面以及胆道癌变。PBM被认为是胰胆管系统中胚胎连接的错误排列，其中末端胆管与腹侧胰腺的一个管道连接。临床表现为间歇性腹痛、复发性急性胰腺炎、黄疸、胆管炎和胆囊癌。在PBM和C…More CBD患者中，原发性胆管结石、急性胆管炎和胆管癌被认为是由胆汁淤积、胰液反流、胆汁和胰液相互反流引起的。此外，胆汁和胰液的相互反流混合很可能在胆道癌变中起重要作用。据报道，在PBM患者中，胆囊上皮细胞增殖活性升高。许多致癌基因和抑癌基因已被确定并与癌变有关，特别是K-ras癌基因和p53抑制基因是PBM胆囊癌变中研究最多的基因。据报道，K-ras突变似乎不是增生所必需的，但可能是癌变的早期事件。p53突变也被报道参与PBM患者胆道上皮的癌变。关键词：肝胆管系统胚胎连接异常，胆囊癌，胆管癌，复发性急性胰腺炎，阻塞性黄疸少

Pancreaticobiliary maljunction (PBM), a congenital anomaly defined as the union of the pancreatic and biliary ducts located outside the duodenal wall, is a recently designated disease entity, and is frequently associated with congenital choledochal cyst (CCBD), which is thought to be different from PBM in embryonic etiology, and variegated clinical features, such as digestive diseases appear in patients with PBM and CCBD.From an analysis of 250 PBM patients (169 with benign hepatobiliary and pancreatic disease and 81 with malignancy) the pathophysiologic and clinical aspects and biliary carcinogenesis in PBM patients were reviewed. PBM is thought to develop as a misarrangement of the embryonic connections in the pancreaticobiliary ductal system, in which the terminal bile duct is joined with one of the ducts of the ventral pancreas.The clinical aspects are intermittent abdominal pain, relapsing acute pancreatitis, jaundice, cholangitis and gallbladder cancer. In patients with PBM and C … More CBD, primary bile duct stones, acute cholangitis and bile duct cancer are considered to result from cholestasis, regurgitation of pancreatic juice, and reciprocal reflux of bile and pancreatic juice. Moreover, the mixture of bile and pancreatic juice due to reciprocal reflux very likely plays an important role in biliary carcinogenesis.In PBM patients, an elevation of cellular proliferation activity in the gallbladder epithelia, was reported. A number of oncogenes and tumor suppressor genes have been identified and implicated in carcinogenesis, and, in particular, the K-ras oncogene and the p53 suppressor gene are the best-studied genes in gallbladder carcinogenesis with PBM. K-ras mutations were reported that did not appear essential for hyperplasia, but may be an early event in carcinogenesis. The p53 mutations were also reported to be involved in carcinogenesis in the biliary epithelium in PBM patients.Key Words: misarrangement of the embryonic connections in pacreaticobiliary ductal system, gallbladder cancer, bile duct cancer, relapsing acute pancreatitis, obstructive jaundice. Less