胰胆管汇合异常与胆道癌的关系

胰胆管汇合异常(pancreaticobiliary maljunction,PBM)为先天发育异常导致胆胰管在十二指肠壁外高位汇合的一种形态学畸形.本研究于经皮经肝胆管引流术(percutaneous transhepatic cholangial drainage,PTCD)或T管造影胰管显影确诊PBM的阻塞性黄疸患者根据病理结果探讨了PBM与胆道癌的关系,以提高胆道癌的临床早期诊断.     

胰腺疾病与异常胰胆管汇合的关系

胰管和胆管在十二指肠壁外汇合并形成一共同通道,其长度超过15mm即为异常的胰胆管汇合(APBJ)。APBJ几乎存在于所有胆总管囊肿的病人中,但非胆总管囊肿病人也可有APBJ。APBJ一般与胆道癌关系密切。另据报道APBJ病人易发生急性胰腺炎,有时还为首发症状。但伴有急性胰腺炎的APBJ病人的临床特点尚不很清楚,其他胰腺疾病与APBJ关系报道很少,而且对APBJ病人胰造影也缺乏分析。本文旨在认清与APBJ相关的胰腺疾病的临床病理特点及胰腺疾病的发病因素,分析APBJ病人胰造影结果,观察相关胰腺疾病的治疗效果,最终建立该疾病的最佳诊疗方…     

胆囊与胆总管重复癌伴胰胆管汇合异常

胰胆管汇合异常(AJPBDS)是先天性胰胆管连接部至十二指肠乳头间存在一较长的共同通道,因该汇合处缺乏 oddi 括约肌而导致了胰液返流至胆总管。先天性胆管扩张症往往伴有 AJPBDS,有认为 AJP-BDS 者胆道系统癌肿的发生率较高,但重复癌却为少见。文献中至今报告有11例伴有 AJPBDS 的胆囊与胆总管重复癌,作者亦报告了1例相同病例。患者女性,64岁,以胆囊肿瘤收入院,无任何自觉症状。超声波检查见胆囊底部二处向腔内突出的息肉样与扁平状肿块;CT 扫描除此外还显示了胆总管扩张,其内亦有软组织肿块;ERCP 所见为胆总管中段腔内肿物伴中度扩张,胆囊未显影,胰胆管汇合部     

胰胆管汇合异常的研究进展

胰胆管汇合异常(pancreaticobiliarymaljunction,PBM)是指在解剖学上胰胆管共同开口于十二指肠乳头之前,形成一过长的共同管,在十二指肠外汇合的一种先天性畸形[1]。在行ERCP的病人中,PBM的发现率约在1.56%~4.5%之间,男女比例约为1∶3,东方多于西方。PBM虽然是一种少见的先天性畸形,但可以导致多种胆胰疾病。一、PBM的诊断标准和分型1991年,日本胰胆管汇合异常学会提出的PBM诊断标准为:胆管与胰管在十二指肠壁外汇合;共同管大于15mm。某些后天因素如十二指肠乳头炎、胆管或者胰管结石、肿瘤等引起的异常不在此列。对于共同通道的长…     

胰胆管汇合异常的临床研究进展

胰胆管汇合异常(PBM)是一种临床罕见的先天性畸形,是指胰管与胆管在十二指肠壁外汇合。PBM因其与众多胆胰疾病的发生密切相关而被广泛关注。笔者就其分型、病理及病理生理机制、早期诊断与治疗等方面的诊治进展做一综述。     

胰胆管合流异常与胆道疾病的关系

我院自1980-1994年间经影像学检查证实胰胍管合流异常28例．其中合并胆总管囊状扩张15例，柱状扩张7例，胆囊癌变4例，胆总管癌变1例。本文就胰胆管合流异常的慨念，分型及与胆道疾患发病之间的关系，病理和病因方面进行了讨论，井对胰胆管台流异常并发胆道疾患时的特殊处理原则进行探讨。     

胰胆管汇合异常病理解剖与胰胆系疾病关系研究

多层螺旋CT及图像后处理技术、MRI等的广泛应用,为胰胆管汇合部解剖学研究提供了重要手段.通过胰胆管汇合异常与胆道系统病变关系研究,可以提高对胆道系统病的发病机理、病理过程的认识,为预防性治疗提供依据.本文对胰胆管汇合异常的定义、分型、病理解剖及其与胆系疾病的关系、临床治疗进行综述.     

胰胆管合流异常与胆道肿瘤

胰胆管合流异常(anomalous Dancreaticobiliary ductal union，APBDU)于1969年由Babbitt等^[1]首次报道，随着内镜逆行性胆管胰管造影术(endoscopic retrograde cholangiopancreatography，ERCP)和磁共振胰胆管成像(magnetic resonance cholangiography，MRCP)等影像学检查的广泛应用，APBDU检出率有增多趋势。APBDU与     

伴有胰胆管系统异常汇合部的成人胆管癌

胰胆管系统异常汇合部（AJPBDS）是指胰胆管汇合部位于十二指肠壁外的一种先天性异常。由于在汇合部缺乏括约肌，可导致两个方向的回流，因而可产生各种病理情况如胆管炎、胆囊结石、胆管瘤、胰腺炎和胰腺结石等。AJPBDS是胆总管囊肿的一个致病因素，而最近发现胆道系统的恶性肿瘤的发生率也很高。本研究的目的是阐明AJPBDS的临床病理特征，并制定治疗与这异常有关的恶性肿瘤的外科方法。病人和方法作者收集了该院自1977年1月至1996年6月间的38例患AJPBDS病人，其中28例有胆道扩张（胆总管囊肿）．有14例经初步会诊或术中证实为胆…     

胰胆管合流异常与胰胆管疾病的关系

自Babbitt[1]1969年首次报道胰胆管合流异常(anomalouspancreaticobiliary ductal uion,APBDU)以来,人们发现APBDU与许多胆道及胰腺疾病密切相关。近年来,随着MRCP、ER-CP、PTC等影像学技术的发展和广泛应用,有关的报道逐渐增多[2],对APBDU又有了更进一步的认识。APBDU的基本概念解剖学上APBDU是指胰胆管共同通道在十二指肠壁外合流,造成共同通道过长,使十二指肠乳头括约肌的作用不能影响到整个合流部,胆汁、胰液相互逆流而引起胆道及胰腺的各种疾病。APBDU分为两型:①P-C型,即胰管注入胆总管型;②C-P型,即胆总管注入胰…     

Clinicopathology of pancreaticobiliary maljunction: relationship between alterations in background biliary epithelium and neoplastic development

Background/Purpose Between 1988 and 2003, 38 patients underwent biliary resection for pancreaticobiliary maljunction (PBM). We reviewed the histopathologic findings for the surgically resected specimens to compare the clinical and pathologic features and assess the relationship between changes in the background biliary epithelium and the development of neoplasms.Methods Papillary hyperplasia (PHP) seen in the biliary epithelium of patients with PBM, was classified into grades 0–III in the gallbladder and grades 0–II in the extrahepatic bile duct, according to the extent, and was assessed for links with tumors in the same specimens.Results The incidence of gallbladder carcinoma was 13/21 in grades I–II, versus 0/16 in grade III, while the incidence of bile duct carcinoma was 4/20 in grade I versus 0/5 in grade II. Furthermore, these incidences for patients below age 50 years and age 50 or older were 1/18 versus 12/20, and 0/14 versus 6/17, respectively.Conclusions PHP of the biliary epithelium in PBM patients is an important precursor lesion, especially for gallbladder cancer, and the risk becomes greater with age, regardless of the type of pancreatobiliary junction (PBJ) and its location in the biliary tract.     

合并胰胆管合流异常的急性胆源性胰腺炎32例分析

目的 通过磁共振胰胆管成像检查评价成人胰胆管合流异常并探讨其和急性胆源性胰腺炎发生的关系.方法 对照观察伴有胰胆管合流异常与不伴胰胆管合流异常之成人急性胆源性胰腺炎患者153例,比较其保守治疗前后的各项肝功能指标的差别.结果 通过磁共振胰胆管成像检出的32例急性胆源性胰腺炎患者合并胰胆管合流异常,与其余121例不伴胰胆管合流异常的成人急性胆源性胰腺炎患者比较.保守治疗后胰胆管合流异常(PBM)组和不伴胰胆管合流异常(NPBM)组的TB、ALT、AST、ALP、GGT都明显降低,其中ALT、AST、GGT的变化差异有统计学意义(P＜0.05).PBM组的ALT、AST和GGT都高于NPBM组,差异有统计学意义(P＜0.05).结论 胰胆管合流异常是导致急性胆源性胰腺炎的重要原因之一,MRCP作为一种无创的胆胰管道系统成像技术对于胰胆管合流异常的研究是一种安全、可靠的检查方法.     

Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease

We report a case of advanced bile duct carcinoma arising in a 15-year-old female with pancreaticobiliary maljunction and congenital biliary cystic disease. Pancreaticoduodenectomy and partial resection of the liver was performed. Surgical and histopathological findings indicated advanced tubular adenocarcinoma, classified as final stage IVb according to the General rules for surgical and pathological studies on cancer of the biliary tract proposed by the Japanese Society of Biliary Surgery, 5th edition, and stage IV according to the American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC), 6th edition. She underwent chemotherapy with gemcitabine HCl after discharge. She died of cachexia 14 months after the surgery. Although it is well known that biliary malignancies arise frequently in patients with pancreaticobiliary maljunction, it is uncommon for advanced bile duct carcinoma to occur in a 15-year-old female. We should pay attention to the possibility of biliary malignancy in patients with pancreaticobiliary maljunction and congenital biliary cystic disease, even when the patients are juveniles.     

Spontaneous necrosis of gallbladder carcinoma in patient with pancreaticobiliary maljunction

While gallbladder carcinoma is occasionally associated with pancreaticobiliary maljunction, spontaneous necrosis of carcinoma is extremely rare. We herein present a case of spontaneous necrosis of gallbladder carcinoma associated with direct invasion of viable cancer cell nests to the muscularis propria and subserosal layer located beneath the primary nodules. A 65-year-old Japanese man was admitted to a local hospital, complaining of repeated discomfort in the right hypochondrium. Ultrasonography and computed tomography scanning revealed cholecystitis associated with gallstones. Cholecystectomy was performed, and operative cholangiography demonstrated pancreaticobiliary maljunction. The resected gallbladder showed multiple mixed stones filled with necrotic debris and bile sludge. Scrutiny of the mucosal surface revealed multiple small necrotic nodules in the fundus, which were histologically confirmed to be necrotic remnants of a cancerous glandular structure. Small nests of papillary adenocarcinoma were found beneath the nodules in the muscularis propria and in the venous structure located in the connective tissues next to the divided margin of the gallbladder bed. Resection of S4a and S5 of the liver and resection of the extrahepatic bile duct was then performed to remove the remaining cancerous tissues and/or micrometastasis in the liver and bile duct. The biliary tree was reconstructed with a hepaticoduodenostomy. No cancer nests or any precancerous lesions were found in the additionally resected specimens. This case indicates a unique morphological feature of gallbladder carcinoma associated with pancreaticobiliary maljunction, which provides some insight into the pathogenesis of spontaneous necrosis of gallbladder carcinoma. Received: August 8, 2000 / Accepted: October 26, 2000     

Recent advances in pancreaticobiliary maljunction

The purpose of this review is to evaluate our current knowledge of the embryologic etiology of pancreaticobiliary maljunction (PBM), its diagnosis, clinical aspects, and treatment, and to clarify the mechanisms of PBM involvement in carcinogenesis. Although the embryologic etiology of PBM still awaits clarification, an arrest of the migration of the common duct of the biliary and pancreatic ducts inwards in the duodenal wall has hitherto been speculated to result in a long common channel in PBM. However, we propose the hypothesis that the etiology of PBM is caused by a disturbance in the embryonic connections (misarrangement) of the choledochopancreatic duct system in the extremely early embryo. That is, PBM is an anomaly caused by a misarrangement whereby the terminal bile duct joins with a branch of the ventral pancreatic duct system, including the main pancreatic duct. PBM is frequently associated with congenital bile duct cyst (CCBD). However, these two anomalies are thought to have different embryonic etiologies. The diagnostic criteria for PBM are the radiological and anatomical detection of the extramural location of the junction of the pancreatic and biliary ducts in the duodenal wall. However, in PBM patients with a short common duct (less than 1 cm in length), detection of the extramural location is difficult. The clinical features of PBM are intermittent abdominal pain, with or without elevation of pancreatic enzyme levels; and obstructive jaundice, with or without acute pancreatitis, while the clinical features of PBM patients with CCBD are primary bile duct stone and acute cholangitis. The optimum approach for the treatment of PBM is the prevention of the reciprocal reflux of bile and pancreatic juice in the pancreas and the bile duct system. To achieve these aims, the surgical approach is most effective, and complete biliary diversion procedures with bile duct resection (for example, choledochoduodenostomy or choledochojejunostomy of the Roux-en-Y type) are most useful. Recently, it has been recognized that the development of biliary ductal carcinoma is associated with PBM. That is, the development of gallbladder cancer occurs frequently in PBM patients without CCBD, and bile duct cancer originating from the cyst wall also occurs in PBM patients with CCBD. It is speculated that the pathogenesis of the bile duct or gallbladder cancer in PBM patients involves the reciprocal reflux of bile and pancreatic juice. Investigations of epithelial cell proliferation in the gallbladder of PBM patients, and of K-ras mutations and p53 suppressor gene mutations, loss of heterozygosity of p53, and overexpression of the p53 gene product in gallbladder cancer and noncancerous lesions in PBM patients have been carried out in various laboratories around the world. The results support the conclusion that PBM is a high risk factor for the development of bile duct carcinoma. Received: March 12, 2001 / Accepted: April 16, 2001     

Biliary carcinogenesis in pancreaticobiliary maljunction

The coexistence of bile duct carcinoma in choledochal cysts is well known. With large number of cases of congenital bile duct dilatation and pancreaticobiliary maljunction (PBM), it is now apparent that gallbladder carcinoma is more frequent in PBM without bile duct dilatation. The incidence of Gallbladder cancer in choledochal cyst and bile duct cancer in PBM, regardless of the presence of bile duct dilatation is significantly higher than that in control patients with biliary cancer but without PBM. A recent survey shows that the incidence of bile duct cancer in PBM without dilatation is equivalent to that of gallbladder cancer and bile duct cancer in patients with choledochal dilatation. Pathology in PBM is a result of reflux of pancreatic juice and stasis of the mixture in the biliary system. Carcinogenic factors thus present in the biliary contents induce epithelial changes in the biliary tract. In the biliary contents, activated pancreatic enzymes and secondary or deconjugated bile acids are markedly increased and they irritate the biliary mucosa. Mutagens are proven to form in the bile of PBM. In the epithelia of PBM, hyperplasia, metaplasia, and dysplasia are often found, and proliferative activity is increased; furthermore, K-ras gene mutation and overexpression of p53 protein are demonstrated. As for treatment of PBM, whether it is symptomatic or not, an operative procedure, is necessary, to prevent carcinogenic changes in the gallbladder and bile duct. Regardless of whether dilatation is present or not, total excision of the extrahepatic bile duct, along with gallbladder, is the treatment of choice, followed by hepaticojejunostomy or hepaticoduodenostomy.