Duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲ a hilar cholangiocarcinoma

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a singlelayer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow- up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.     

Parapapillary choledochoduodenal fistula associated with cholangiocarcinoma

Parapapillary choledochoduodenal fistula is a rare disorder. We herein report a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma. A 61‐year‐old woman was admitted to our hospital for further examination of a liver tumor. She had no clinical symptoms, but computed tomography scans showed an irregularly contoured liver tumor which was histologically confirmed to be adenocarcinoma, by a needle biopsy examination. Duodenal fiberscopy revealed a fistula orifice 1.0 cm proximal to the orifice of the papilla of Vater, and endoscopic retrograde cholangiography through the fistula showed a communication to the common bile duct. Hypotonic duodenography demonstrated reflux of contrast material into the choledochoduodenal fistula. The bile sample collected from the common bile duct showed extremely high levels of pancreatic enzymes, including amylase, phospholipase‐A2, and elastase‐I. Furthermore, Helicobacter DNA was detected in bile by polymerase chain reaction (PCR) analysis. This experience suggests to us that parapapillary choledochoduodenal fistula may be a risk factor for biliary tract carcinoma, and surgical management is the treatment of choice for this rare condition, even when the patient has no significant clinical symptoms.     

A case of primary adenosquamous carcinoma of the liver with formation of colonic fistula]

Primary adenosquamous carcinoma of the liver is generally considered as an extremely rare subtype of cholangiocarcinoma. It has been reported mostly in a form of case studies. As far as we know, there was only one case report on tumor related with biliary fistula. Recently, we experienced a case of primary adenosquamous carcinoma of liver with a formation of tumor-colonic fistula. A 54-year-old man was transferred to our hospital due to liver mass detected by abdominal ultrasonogram. Dynamic computed tomogram of liver showed a large irregular hypodense mass without rim enhancement in right lobe of liver and also suggested a fistula formation between the tumor and hepatic flexure of right colon. Colonoscopic examination showed a large colonic wall defect in hepatic flexure and a friable, nodular mucosa around the defected colonic wall. Extended right lobectomy and right hemicolectomy were done. Microscopically, the tumor was composed of squamous cell carcinoma mainly with foci of the adenocarcinoma component.     

Biliary dysplasia as a marker of cholangiocarcinoma in primary sclerosing cholangitis

BACKGROUND/AIMS: Indentification of biliary dysplasia in a primary sclerosing cholangitis (PSC) liver biopsy may indicate developing cholangiocarcinoma. The objectives were to determine whether biliary dysplasia can be recognised reproducibly in PSC and to compare the frequency in cases with and without cholangiocarcinoma. METHODS: Liver biopsies from 26 PSC cases with concurrent or subsequent cholangiocarcinoma (within 2 years) were assessed for biliary dysplasia independently by three liver pathologists. This was done in two stages: initially, without agreement on criteria, and subsequently after such agreement. Liver biopsies from 60 PSC cases without cholangio-carcinoma were also assessed. RESULTS: Reproducibility for biliary dysplasia without prior agreement on criteria was only marginally better than random (kappa=0.129). In contrast, after prior agreement on criteria, reproducibility was moderate (kappa=0.44). Biliary dysplasia was agreed to be present by all three pathologists in 23% and 19% of biopsies in the first and second round, respectively, from patients with cholangiocarcinoma, but in none of the patients without cholangiocarcinoma. CONCLUSION: Criteria for biliary dysplasia can be agreed and the entity recognised in liver biopsies. The strong association of biliary dysplasia with cholangiocarcinoma in PSC suggests use of dysplasia as a marker for current or developing malignancy.     

The value of biliary fibronectin for diagnosis of cholangiocarcinoma

BACKGROUND/AIMS: The fibronectin in various body fluids was reported to be useful in the differential diagnosis of benign and malignant diseases. We conducted this study to assess the diagnostic value of biliary fibronectin. METHODOLOGY: Bile was collected from patients with gallstones (n = 62), benign biliary stricture (n = 5) and cholangiocarcinoma (n = 28). All patients with gallstones were complicated with cholangitis. The absolute biliary level of fibronectin was determined by an immunoenzymatric assay and divided by the biliary concentration of bile acids to calculate the relative concentration. RESULTS: The relative concentration of biliary fibronectin was significantly different among the patients with benign biliary obstruction, gallstone and cholangiocarcinoma (7.1 +/- 14.3 vs. 350.5 +/- 1535.4 vs. 180592.6 +/- 917236.2, p < 0.05). With a cut-off value of 40 ng/mumol, its sensitivity for diagnosing cholangiocarcinoma was 0.57, and specificity, 0.79. CONCLUSIONS: As bile may be concentrated or diluted to a various extent after biliary obstruction, the relative concentration of biliary fibronectin is more useful than the absolute concentration in differentiating the causes of biliary obstruction. In addition to the presence of biliary cancer, biliary fibronectin was also increased in response to biliary inflammation, which limits its use as a tumor marker of cholangiocarcinoma.     

Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas

Recently, attention has been drawn to papillary neoplasm of the pancreatobiliary systems. In the pancreas, the disease entity of intraductal papillary mucinous neoplasm (IPMN-P) is widely recognized. In contrast, the pathological characteristics of biliary papillary tumors, such as biliary papilloma(tosis) and papillary cholangiocarcinoma, have not yet been well documented. In this study, we compared the pathological features and post-operative prognosis among biliary papillary tumors (10 cases of biliary papilloma(tosis) and 22 cases of papillary cholangiocarcinoma), conventional non-papillary cholangiocarcinoma (15 cases), and IPMN-P (31 cases). Macroscopically, all biliary papillary tumors were characterized by the prominent intraductal papillary proliferation, and macroscopic mucin-hypersecretion was seen in 9 of 32 cases (28%). Histologically, biliary papillary tumors consisted of three types of tumor cells (pancreaticobiliary, intestinal and gastric types), whereas only the pancreaticobiliary type was observed in non-papillary cholangiocarcinoma. Immunohistochemically, biliary papillary tumors were characterized by the common expression of MUC2, CDX2 and cytokeratin 20. In addition, biliary papillary tumors could be associated with two types of invasive lesions: tubular adenocarcinoma (9 cases) and mucinous carcinoma (5 cases). Patients with tubular adenocarcinoma had a poor prognosis compared to non-invasive papillary tumor or papillary tumor with mucinous carcinoma. These pathological characteristics and the survival status of biliary papillary tumors were different from those of non-papillary cholangiocarcinoma, and rather closely resembled those of IPMN-P. In conclusion, biliary papillary tumors may be the biliary counterpart (intraductal papillary neoplasm of the bile duct) of IPMN-P.     

Biliary papillary hyperplasia with clonorchiasis resembling cholangiocarcinoma

Infection by the liver fluke Clonorchis sinensis is very common in the Far East. It causes low grade inflammatory changes and proliferation in the biliary tree. Initially there is desquamation of the biliary epithelium, followed by hyperplasia and adenomatous proliferation. Cholangiocarcinomas are potential long term complications. We present a case of biliary papillary hyperplasia with clonorchiasis resembling cholangiocarcinoma in a 69-yr-old Korean man. Early recognition of biliary hyperplasia and treatment of Clonorchis sinensis is important to prevent development of cholangiocarcinoma, especially in the Far East.     

内镜下金属支架引流术治疗肝门部胆管癌和肝外恶性胆道梗阻临床观察

临床上恶性胆道梗阻性疾病预后较差。对于不能手术切除者,通常选择内镜下置人胆道支架以解除梗阻,然而该技术对进展期肝门部肿瘤的疗效报道不一。目的：探讨内镜下金属支架引流术对肝门部胆管癌和肝外恶性胆道梗阻的疗效和并发症发生情况。方法：纳入上海交通大学附属第一人民医院2006年6月～2009年6月收治的82例接受ERCP下置入自膨式金属胆道支架引流治疗的恶性胆道狭窄患者,根据病变部位分为肝门部胆管癌组和肝外恶性胆道梗阻组,对其ERCP参数和术后6个月随访记录进行回顾性分析,并分析随访期间急性胆管炎发生的危险因素。结果：两组支架置入成功率均为100％。与肝外恶性胆道梗阻组相比,肝门部胆管癌组术后1周总胆红素降低显效率较低,术后6个月内急性胆管炎发生率增高,初次发生时间提前,支架再狭窄率增高（P=0．000）。ERCP术中括约肌切开为随访期间发生急性胆管炎的危险因素（P=0．004,OR：8．196）。结论：内镜下金属支架引流术对肝门部胆管癌的疗效不及肝外恶性胆道梗阻,且更易早期发生急性胆管炎和支架再狭窄,术中括约肌切开可增加术后急性胆管炎的发生风险。     

Indications for non-transplant surgery in primary sclerosing cholangitis

Primary sclerosing cholangitis (PCS) is a progressive disease leading to secondary biliary cirrhosis. Patients are at increased risk of developing cholangiocarcinoma, which is usually diagnosed at an advanced stage. Treatment of PCS includes medical therapy, endoscopic biliary dilation, percutaneous transhepatic stenting, extrahepatic biliary resection and liver transplantation. The most effective management of primary sclerosing cholangitis before the onset of cirrhosis remains unclear. Non-transplant surgical procedures have a limited but defined role in patients with PCS. Resection of the extrahepatic biliary tree in symptomatic non-cirrhotic patients improves hyperbilirubinaemia and prolongs both transplant-free and overall survival when compared with non-operative dilation and/or stenting. Surgical resection may also definitively establish or exclude a diagnosis of cholangiocarcinoma in patients with dominant extrahepatic or perihilar strictures. Extrahepatic bile duct resection may also reduce the risk of cholangiocarcinoma. Extrahepatic biliary resection should be considered in selected non-cirrhotic patients with symptomatic biliary obstruction and dominant extrahepatic and/or perihilar strictures. Those patients in whom cholangiocarcinoma is suspected should also undergo resection.     

Cholangiocarcinoma and malignant bile duct obstruction: A review of last decades advances in therapeutic endoscopy

In the last decades many advances have been achieved in endoscopy, in the diagnosis and therapy of cholangiocarcinoma, however blood test, magnetic resonance imaging, computed tomography scan may fail to detect neoplastic disease at early stage, thus the diagnosis of cholangiocarcinoma is achieved usually at unresectable stage. In the last decades the role of endoscopy has moved from a diagnostic role to an invaluable therapeutic tool for patients affected by malignant bile duct obstruction. One of the major issues for cholangiocarcinoma is bile ducts occlusion, leading to jaundice, cholangitis and hepatic failure. Currently, endoscopy has a key role in the work up of cholangiocarcinoma, both in patients amenable to surgical intervention as well as in those unfit for surgery or not amenable to immediate surgical curative resection owing to locally advanced or advanced disease, with palliative intention. Endoscopy allows successful biliary drainage and stenting in more than 90% of patients with malignant bile duct obstruction, and allows rapid reduction of jaundice decreasing the risk of biliary sepsis. When biliary drainage and stenting cannot be achieved with endoscopy alone, endoscopic ultrasound-guided biliary drainage represents an effective alternative method affording successful biliary drainage in more than 80% of cases. The purpose of this review is to focus on the currently available endoscopic management options in patients with cholangiocarcinoma.     

Preoperative biliary drainagein hilar cholangiocarcinoma:Whenandhow?简

Hilar cholangiocarcinoma is a tumor of the extrahepatic bile duct involving the left main hepatic duct, the right main hepatic duct, or their confluence. Biliary drainage in hilar cholangiocarcinoma is sometimes clinically challenging because of complexities associated with the level of biliary obstruction. This may result in some adverse events, especially acute cholangitis. Hence the decision on the indication and methods of biliary drainage in patients with hilar cholangiocarcinoma should be carefully evaluated. This review focuses on the optimal method and duration of preoperative biliary drainage (PBD) in resectable hilar cholangiocarcinoma. Under certain special indications such as right lobectomy for Bismuth type IIIA or IV hilar cholangiocarcinoma, or preoperative portal vein embolization with chemoradiation therapy, PBD should be strongly recommended. Generally, selective biliary drainage is enough before surgery, however, in the cases of development of cholangitis after unilateral drainage or slow resolving hyperbilirubinemia, total biliary drainage may be considered. Although the optimal preoperative bilirubin level is still a matter of debate, the shortest possible duration of PBD is recommended. Endoscopic nasobiliary drainage seems to be the most appropriate method of PBD in terms of minimizing the risks of tract seeding and inflammatory reactions.     

Cholangiocarcinoma

Cholangiocarcinomas are rare malignant tumors composed of cells that resemble those of the biliary tract. On the basis of their anatomic location, cholangiocarcinomas can be classified as intrahepatic, extrahepatic and hilar tumors. For reasons that are not clear, the incidence of cholangiocarcinoma is increasing globally. Established risk factors, including conditions associated with chronic biliary tract inflammation, account for a small proportion of cases. Additional risk factors such as cirrhosis, infection with hepatitis B virus and hepatitis C virus are now becoming recognized. The diagnosis of cholangiocarcinoma requires the integration of clinical information, imaging studies of the hepatic parenchyma and biliary tract, tumor markers, and histology. In terms of the treatment options for cholangiocarcinoma, surgery can be curative, although few patients are candidates for surgery. Palliative biliary decompression can provide symptomatic relief. Advances in MRI and positron-emission tomography scanning, identification of new tumor markers, improved utility of biliary cytology, and the use of photodynamic therapy for adjunct treatment are all expected to enhance the diagnosis, evaluation and management of cholangiocarcinoma.     

Preoperative biliary drainage before resection for cholangiocarcinoma (Pro)

Three types of preoperative biliary drainage (BD): percutaneous transhepatic (PTBD), endoscopic (EBD), and endoscopic nasobiliary (ENBD) can be indicated before resection of cholangiocarcinoma. However, three randomized controlled trials (RCTs) have revealed that preoperative PTBD does not improve perioperative results. Other RCTs have revealed that preoperative EBD for malignant obstructive jaundice has no demonstrable benefit and after EBD for hilar cholangiocarcinoma there are highly developed infectious complications. Most patients with distal cholangiocarcinoma undergo pancreatoduodenectomy (PD) without preoperative BD. However, no RCTs have been performed to clarify the safety of major hepatectomy without preoperative BD for cholestatic patients with hilar cholangiocarcinoma. Furthermore, preoperative intrahepatic segmental cholangitis is a prognostic factor in the outcome of major hepatectomy for biliary cancer. Preoperative BD has another purpose in the preoperative management of patients with hilar cholangiocarcinoma. Selective cholangiography via ENBD and/or PTBD catheters provides precise information about the complicated segmental anatomy of the intrahepatic bile ducts and extent of cancer along the separated segmental bile ducts, which contributes toward designing a type of resective procedure. RCTs in biliary cancer patients undergoing major hepatectomy have revealed that bile replacement during external biliary drainage and perioperative synbiotic treatment can prevent postoperative infectious complications. Although preoperative EBD increases the risk of cholangitis, major hepatectomy combined with preoperative biliary drainage, preferably PTBD and/or ENBD, followed by portal vein embolization has been established as a safer management strategy for perihilar cholangiocarcinoma.     

Clinical diagnosis and staging of cholangiocarcinoma

Cholangiocarcinoma is the most frequent biliary malignancy. It is difficult to diagnose owing to its anatomic location, growth patterns and lack of definite diagnostic criteria. Currently, cholangiocarcinoma is classified into the following types according to its anatomic location along the biliary tree: intrahepatic, perihilar or distal extrahepatic cholangiocarcinoma. These cholangiocarcinoma types differ in their biological behavior and management. The appropriate stratification of patients with regard to the anatomic location and stage of cholangiocarcinoma is a key determinate in their management. Staging systems can guide this stratification and provide prognostic information. In addition, staging systems are essential in order to compare and contrast the outcomes of different therapeutic approaches. A number of staging systems exist for cholangiocarcinoma-several early ones have been updated, and new ones are being developed. We discuss the emerging diagnostic criteria as well as the different staging systems for cholangiocarcinoma, and provide a critical appraisal regarding these advances in biliary tract malignancies.     

Cholangiocarcinoma arising from preexisting biliary hamartoma of liver--report of a case

We describe the case of a 72-year-old asymptomatic man with a cholangiocarcinoma arising from a biliary hamartoma, also referred to as "von Meyenburg's complex". The patient was clinically diagnosed as having a cystadenocarcinoma, but the tumor had already been present as a uniformly low-density area on computed tomography taken four years previously, as revealed by retrospective examination of the computed tomography films that had been taken annually after surgery for pulmonary emphysema. The low-density area had continued to enlarge year after year, and a high-density area was observed to have emerged inside the low-density area on computed tomography. Histopathological examination demonstrated that the high-density area corresponded to the cholangiocarcinoma and the low-density area to a biliary hamartoma. This is the first case in which it was possible to confirm the presence of cholangiocarcinoma inside a biliary hamartoma that had continued to increase in size.     

Surgical management of cholangiocarcinoma

Biliary tract cancer affects approximately 7500 Americans each year. Tumors arising from the gallbladder are the most common; those of bile duct origin, or cholangiocarcinoma, are less frequently encountered, constituting approximately 2% of all reported cancers. Although cholangiocarcinoma can arise anywhere within the biliary tree, tumors involving the biliary confluence (i.e., hilar cholangiocarcinoma) represent the majority, accounting for 40 to 60% of all cases. Twenty to 30% of cholangiocarcinomas originate in the lower bile duct, and approximately 10% arise within the intrahepatic biliary tree and will present as an intrahepatic mass. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. For all patients with intrahepatic cholangiocarcinoma and nearly all patients with hilar tumors, complete resection requires a major partial hepatectomy. Distal cholangiocarcinomas, on the other hand, are treated like all periampullary malignancies and typically require pancreaticoduodenectomy. Most patients with cholangiocarcinoma present with advanced disease that is not amenable to surgical treatment, and even with a complete resection, recurrence rates are high. Adjuvant therapy (chemotherapy and radiation therapy) has not been shown clearly to reduce recurrence risk.     

Cholangiocarcinoma: recent progress. Part 2: molecular pathology and treatment

Part 2 of this review discusses DNA damage in biliary epithelial cells in the development of cholangiocarcinoma, alterations in cell kinetics of biliary epithelial cells, biliary epithelial mitoinhibition, and apoptosis that includes the role of Bcl-2, transforming growth factor-beta, telomerase activities and deregulation of Ras and p53, cancer-associated antigens in cholangiocarcinoma, precancerous lesions, stroma formation and angiogenesis, cancer invasion, cell-cell and cell-matrix interactions, and the mechanism of evasion from immune surveillance. These discussions are followed briefly by treatments such as photodynamic therapy, and surgical approaches comparing resection and liver transplantation.     

47例囊性肝棘球蚴病合并胆管瘘病解剖特征的临床资料分析

目的 初步探讨囊性肝棘球蚴病合并胆管瘘病的临床分型标准及意义。 方法 回顾性研究2000年1月至2005年3月，收治并行外膜内完整摘除术治疗囊性肝棘球蚴病合并胆管瘘病47例，术中观察胆管瘘不同的解剖特点，术后观察疗效。 结果与结论 47例患者术后恢复顺利，无残腔感染及胆管瘘等并发症。总结胆管瘘不同的解剖特点，初步提出囊性肝棘球蚴病合并胆管瘘病的3个临床分型标准，即：根据囊性肝棘球蚴病发病部位分为中央型及外周型，根据胆管与囊肿解剖关系分为侧瘘型、直入型及贯通型，根据肝棘球蚴囊固态内容物与胆道的关系分为破入胆道型及未破入胆道型。按此标准，可明确地表述囊性肝棘球蚴病合并胆管瘘病的情况，对临床外科具有参考意义。     

Metal stents: a bridge to surgery in hilar cholangiocarcinoma

BackgroundObstructive jaundice in patients with hilar cholangiocarcinoma is a known risk factor for hepatic failure after liver resection. Plastic stents are most widely used for preoperative drainage. However, plastic stents are known to have limited patency time and therefore, in palliative settings, the self‐expanding metal stent (SEMS) is used. This type of stent has been shown to be superior because it allows for rapid biliary decompression and a reduced complication rate after insertion. This study explores the use of the SEMS for biliary decompression in patients with operable hilar cholangiocarcinoma.MethodsA retrospective evaluation of a prospectively maintained database at a tertiary hepatobiliary referral centre was carried out. All patients with resectable cholangiocarcinoma were recorded.ResultsOf 260 patients referred to this unit with cholangiocarcinoma between January 2008 and April 2012, 50 patients presented with operable cholangiocarcinoma and 27 of these had obstructive jaundice requiring stenting. Ten patients were initially treated with SEMSs; no stent failure occurred in these patients. Seventeen patients initially received plastic stents, seven of which failed in the interval between stent placement and laparotomy. These stents were replaced by SEMSs in four patients and by plastic stents in three patients. Median time to laparotomy was 45 days and 68 days in patients with SEMSs and plastic stents, respectively.ConclusionsSelf‐expanding metal stents provide adequate and rapid biliary drainage in patients with obstruction caused by hilar cholangiocarcinoma. No re‐interventions were required. This probably reflects the relatively short interval between stent placement and laparotomy.