成人胰胆管合流异常患者胆囊黏膜病理形态观察

目的探讨成人胰胆管合流异常患者胆囊黏膜病理形态变化。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胆囊疾病患者中的胆囊黏膜增殖细胞核抗原染色切片,以比较其病理形态的差别。结果3年内通过术中胆道造影及内镜逆行胰胆管造影检查检出13例胆囊疾病患者合并胰胆管合流异常,与随机抽取的13例经以上检查明确不伴胰胆管合流异常之成人胆囊疾病患者比较,胰胆管合流异常组增殖细胞核抗原阳性率为(31.13±4.22)%;而对照组则为(23.78±6.57)%;精确卡方检验显示胰胆管合流异常组与对照组之间胆囊黏膜增殖性差异有统计学意义(P<0.01)。结论合并胰胆管合流异常之成人胆囊疾病患者胆囊黏膜增殖性高于不伴胰胆管合流异常之成人胆囊疾病患者。     

重视胰胆管合流异常的处理

近年来,由于影像学及电生理学的长足进步,胰胆管合流异常与胆胰疾病间的关系日益受到临床医师们的重视,并已取得了一定的研究成果。胰胆管合流异常(anomalous arrange-ment of pancreaticobiliary duct,APBD)可导致多种胆胰疾病已得到临床证实,尤其是与先天性胆总管囊肿、胆管结石和急、慢性胰腺炎的发病有一定的关系,且愈来愈受到重视。但因其位置特殊,给临床研究带来较多困难,极易导致临床上的误诊和误治,仍是亟待解决的难题之一。一、APBD的概念1.胰胆管汇合部的正常解剖:生理情况下,胆、胰管在十二指肠壁内近乎平行走行,在十二指肠…     

胰胆管合流异常和胆道疾病

胰胆管合流异常和胆道疾病上海第二医科大学附属仁济医院（２００００１）季福，施维锦由于内镜逆行胰胆管造影（ＥＲＣＰ）和经皮经肝胆管穿刺造影（ＰＴＣ）在临床上的广泛应用，发现胰胆管合流异常（ＡＪＰＢＤ）的病例增多。自从１９６９年Ｂａｂｉｔｔ首先提出ＡＪＰ...     

胰胆管合流异常三例

胰胆管汇合异常是指胆总管与主胰管在十二指肠壁外汇合的一种少见的解剖异常 ,可引起许多胆胰系统疾病。我院 2 8例术后胆道造影患者中 ,有 8例出现主胰管显影 ,其中 3例清晰显示胆总管与主胰管在十二指肠壁外汇合 ,现对其作一报道。病历摘要例 1:男 ,5 7岁。无痛性黄疸 10ｄ以“肝门部胆管癌”住院。术中见 :肿瘤位于肝门部 ,直径 4ｃｍ ,固定。以胆道探子扩张右肝管 ,置入 6ｃｍ长镍钛记忆合金胆道支架 ,胆总管内置 1根引流管 ,肿瘤表面以钛夹标志。术后 10ｄ经胆管引流管行胆道造影 ,见胆总管直径 0 .8ｃｍ ,肝内胆管直径 1.0ｃｍ ,主胰…     

胰胆管合流异常动物模型的建立

目的建立胰胆管合流异常的动物模型。方法选用健康杂种猫10只。术前禁食12 h,3.5%戊巴比妥钠麻醉后,取上腹正中切口约6 cm切开各层至腹腔。于胆总管入十二指肠处旁边,切开胰腺背膜,解剖胰管;靠近十二指肠处分别纵向切开胰管、胆管长约4~6 mm的切口。6-0线间断吻合切口,造成类似人类的胰胆管合流的共同通道。术后20天胆道造影。结果术后动物精神、食欲良好,无萎靡、烦燥等表现,造影显示胰胆管合流共同通道延长。结论本动物模型最接近于人类的胰胆管合流异常生理,优于其他动物模型。     

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。     

先天性胰胆管合流异常20例报告

胰胆管合流异常国内极少报告，临床上常被误诊或漏诊。为提高对本病的诊治水平，现就我院１９８５年以来收治的２０例总结报告如下。１临床资料本组２０例先天性胰胆管合流异常患者中男８例，女１２例。年龄５个月～２４岁，平均７岁零８个月。主要症状有腹痛、发烧、黄疸...     

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     

胰胆管合流异常的病理特征与诊治进展

胰胆管合流异常 (pancreaticobiliary maljunction,PBM)是胰管和胆总管异常汇合的一种先天性畸形 [1 ]。从 1916年 Kizu-mi首先提出 PBM这一概念 ,至 1978年日本学者因此成立“胰胆管合流异常研究会”,PBM已日渐引起国内外广泛关注 ,对PBM基础及临床的研究正逐步深入。1　PBM的病理特征约 75 %的正常人胆总管与主胰管在十二指肠粘膜下汇合成共通管 ,并随着年龄逐渐增长。共通管周围及近端具有 Oddi氏括约肌 ,其中胆总管括约肌部分恒定存在 ,在神经与体液双重因素的调节下可控制胆汁的正常排出 ,亦可防止胰液逆流入胆管。据 1991年日…     

胰胆管合流异常对胆囊损伤的实验研究

胰胆管合流异常(anomalous pancreaticobiliaryductal union,APBDU)时主胰管与胆总管汇合成过长的共同通道,胆汁和胰液互流,引起多种胰胆系统病变.APBDU胆管癌的发生率高于正常人,且发生年龄也比正常人提前.尽管对APBDU进行了大量研究,其机制还未完全明了.以往的研究大多限于临床病例资料回顾,已有的动物模型与人类的生理解剖结构有较大差异~([1]).     

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     

Prophylactic excision of the gallbladder and bile duct for patients with pancreaticobiliary maljunction.

HYPOTHESIS: Pancreaticobiliary maljunction (PBM) is a high-risk factor for biliary tract carcinogenesis because of a continuous reflux of pancreatic juice into the biliary tract. It remains to be disclosed whether we should perform prophylactic excision of gallbladders and bile ducts. DESIGN: A person-year method. SETTING: A university hospital. PATIENTS: We studied 68 patients with PBM treated between August 1, 1974, and December 31, 1999. MAIN OUTCOME MEASURES: Relative risks (observed number-expected number ratios) of gallbladder and bile duct carcinomas according to type of bile duct dilation (ie, cystic dilation, diffuse dilation, and nondilation). RESULTS: Observed number-expected number ratios of gallbladder carcinomas were high: 291.3 in 43 patients with cystic dilation, 167.2 in 16 patients with diffuse dilation, and 419.6 in 7 patients with nondilation. Observed number-expected number ratios of bile duct carcinomas were 194.2 in 43 patients with cystic dilation before surgery and 142.8 in 39 patients with cystic dilation after long postsurgical follow-up. All these values were statistically significant (P<.01). CONCLUSIONS: The gallbladder carries a high risk for carcinogenesis in all types of dilation in patients with PBM. The bile duct carcinomas of PBM were exclusively identified by the type of cystic dilation. Prophylactic cholecystectomy should be recommended for all dilation types, and prophylactic excision of bile ducts including cholecystectomy should be performed in patients with PBM and cystic dilation. Complete excision of extrahepatic dilated bile ducts and careful follow-up for carcinogenesis in residual dilated bile ducts should be recommended for patients with PBM and cystic dilation.     

Metachronous double cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction

A 50-year-old Japanese woman complained of abdominal and back pain. Ten years previously she had undergone cholecystectomy, choledochectomy, and Roux-en-Y choledochojejunostomy for gallbladder cancer associated with pancreaticobiliary maljunction without bile duct dilatation. On the present admission, ultrasonography (US) and computed tomography (CT) demonstrated a large mass, 60 mm in size, in the pancreatic tail. Endoscopic retrograde cholangiopancreatography (ERCP) showed obstruction of the main pancreatic duct in the tail of the pancreas and revealed that the pancreatic duct was joined to the bile duct 25 mm above the papilla of Vater. The patient underwent distal pancreatectomy, splenectomy, left adrenalectomy, and partial gastrectomy. Histological examination revealed moderately differentiated ductal adenocarcinoma that had invaded to the proper muscle of the stomach. Double cancer of the gallbladder and pancreas in a patient with pancreaticobiliary maljunction is rare. Although the etiology of cancer of the pancreas associated with pancreaticobiliary maljunction is unclear, we should pay close attention to the pancreas as well as the biliary tract during the long-term follow-up of patients with pancreaticobiliary maljunction after they have undergone a choledochojejunostomy.     

A case report of gallbladder cancer and pancreas cystic neoplasm associated with pancreaticobiliary maljunction

Introduction and importancePancreaticobiliary maljunction (PBM) is a rare congenital anomaly that is frequently associated with carcinoma of the biliary tract. However, there is still no clear evidence that PBM is associated with pancreatic tumors. Here we describe a case of gallbladder cancer and intraductal papillary mucinous neoplasm (IPMN) that is associated with PBM.Case presentationA 72-year-old man underwent a cholecystectomy with hepatectomy (S4a + S5) and regional lymph node dissection for gallbladder adenocarcinoma invading the front lobe branch of the hepatic artery. A pylorus-preserving pancreaticodudenectomy was also performed for pancreatic IPMN.Clinical discussionPresence of mucin type 6 (MUC6) -positive pyloric gland metaplasia in both the dilated pancreatic duct and the gallbladder background mucosa suggests that pancreatic IPMN and gallbladder cancer may have a common phenotypic origin. Additionally, analysis of 41 reported cases of pancreatic cancer associated with PBM revealed that in all metachronous multiple cancer cases, biliary tract cancer preceded the pancreatic cancer with congenital biliary dilatation accompanied by PBM. The analysis also revealed an increased proportion of pancreatic cancer cases with PBM in patients who had not undergone a flow diversion procedure located in pancreatic head.ConclusionWe show an interesting relationship between pancreatic/gallbladder cancer and PBM. More comprehensive evaluations of the whole pancreaticobiliary system in follow-up of patients with PBM is required to understand the full extent of this relationship.     

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.     

Cancer of the gallbladder associated with pancreaticobiliary maljunction without bile duct dilatation in a european patient

A rare case of pancreaticobiliary maljunction (PBM) without dilatation of the biliary tract (DBT) associated with gallbladder carcinoma is described herein. A 62-year-old European woman with a long history of right upper abdominal pain was diagnosed as having PBM without DBT by endoscopic retrograde cholangiopancreatography and other examinations. Excision of the gallbladder and biliary duct with a Roux-en-Y hepaticojejunostomy was performed, and subsequent pathological examination of the surgical specimen showed a well differentiated adenocarcinoma of the gallbladder. She had no clinical symptoms for 58 months postoperatively. PBM allows reflux of pancreatic juice into the biliary tract. Recent findings support the idea that epithelial hyperplasia plays an important role in gallbladder carcinogenesis with PBM, and also support the concept that gene mutations are involved in the carcinogenesis of biliary epithelium in patients with PBM. For these reasons, we advocate that resection of the extrahepatic biliary tract in PBM patients without bile duct dilatation, rather than cholecystectomy alone, is the treatment of choice for preventing bile duct carcinoma. Received for publication on Dec. 15, 1999; accepted on Feb. 22, 2000