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Liver transplantation principally can be considered as a suitable form of treatment for any patient with progressive or end-stage liver disease, when no other standard therapy is available. The two main categories of indications are liver cirrhosis with severe complications from functional deterioration and primary malignancies of the liver.

Operative endoscopy of the biliary system has been employed with ever increasing frequency at the UCLA Hospital during the last three years. In addition to its established value with respect to disclosing unsuspected stones in the bile ducts, choledochoscopy has been of great value in terms of more accurate diagnosis and staging of periampullary and bile duct neoplasms. It has been observed that many biliary tract carcinomas are multicentric in origin and that cholangiography is not adequate to identify small intrahepatic ductal lesions. More than one cell type of bile duct carcinoma may be present in the same patient. Choledochoscopy should be used in addition to the conventional criteria for resectability in all patients with ductal or periampullary carcinoma. Use of this technic will spare some patients needless radical procedures and should improve long-term cure rates by identifying those patients with truly localized disease for curative resections.

An autopsy report of a patient with a hugh biliary cystadenocarcinoma arising in the right lobe of the liver is presented. The tumor had been though to be benign, but peritoneal carcinomatosis developed 18 months after the operation. Autopsy revealed marked peritoneal dissemination of mucinous adenocarcinoma, apparently a result of peritoneal seeding of hepatic cystadenocarcinoma, which was presumed to be triggered by cyst aspiration performed during the operation. Experience indicates that surgical procedures on cystic liver disease should be carried out carefully, considering the possible existence of malignancy.

The registry of digestive tract tumours established for the department of Cote-d'Or (France) was used to study the epidemiological characteristics and the natural history of biliary tract cancers. Age standardised incidence rates for gallbladder cancers were 2.7/100,000 for women and 0.9/100,000 for men. The corresponding rates for extrahepatic bile duct cancers were 0.5/100,000 and 1.7/100,000, and for ampulla of Vater cancer 0.3/100,000 and 0.3/100,000. The three cancers differ in their descriptive epidemiology and should be considered separately in epidemiological analytical investigations. The incidence of each of the three diseases increased with age, and cancers of known histological type were mainly adenocarcinomas. Some gallbladder cancers were undifferentiated or squamous cell carcinomas. There was no significant variation in incidence for gallbladder cancer and extrahepatic bile duct cancer over the eight years of the study. The association with gallstones was frequent in gallbladder cancer: 70.5% compared to 13.0% in other biliary tract cancers (less than 0.001). Although the association of gallbladder cancer with gallstones is frequent, few patients with cholelithiasis experience development of a gallbladder cancer. It is necessary to identify among patients with gallstones a subgroup at high risk of gallbladder cancer in whom prophylactic surgery might be justified. Biliary tract cancers are seldom diagnosed early: lymph nodes or visceral metastases were present in 77% of gallbladder cancers, in 83% of extrahepatic bile duct cancers, and in 55% of ampulla of Vater cancers at the time of diagnosis. The corresponding resectability rates were 46.1%, 11.9%, and 38.9%. The five-year overall survival rates were 2.9% for gallbladder cancer, 0% for extrahepatic bile duct cancer, and 18.3% for ampulla of Vater cancer. The corresponding five-year survival rates after surgery for cure were 10.3%, 0%, and 35.7%. Biliary tract cancer still represent a great therapeutic challenge.

Excerpt Carcinomas of the stomach, biliary tract and pancreas constitute one of the principal causes of death from cancers in both males and females. Not only are these malignant growths among the ...

IN 1948 Longmire and Sanford1 described the technic of intrahepatic cholangiojejunostomy for relief of biliary-tract obstruction due to extensive destruction of the extrahepatic ducts. Since then they have reported 3 additional cases in which the procedure was successfully carried out,2 and the reports of others have brought the total number of cases in which the operation was performed up to 8.3 4 5 6 There have undoubtedly been other cases that have not been reported. The circumstances that warrant the operation rarely arise. Allen,7 Cattell,8 Lahey,9 Cole et al.,10 and Walters and his associates11 have shown that biliary drainage into the intestine can . . .

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Trousseau, 1 in 1856, noted that thrombosis of peripheral veins may be the first clinical indication of the presence of a malignant tumor in some part of the body. This observation has had such recent confirmation as the study of Woolling and Shick. 2 They reported 15 cases of carcinoma that had been unrecognized until after the patients sought medical aid because of symptoms referable to thrombophlebitis. The association of venous thrombosis and carcinoma of the pancreas was carefully investigated in autopsy material by Sproul, 3 who found thrombosis of one or more veins in 14 of 47 patients with carcinoma of the pancreas and in 9 of the 16 patients with carcinoma restricted to the body or tail—a very high incidence. Generations of medical students have been taught, on the basis of these and similar studies, that peripheral thrombophlebitis as a presenting complaint is suggestive of malignant disease in

In recent years with the larger number of patients examined at the Lahey Clinic who require secondary procedures on the biliary tract, we have encountered amputation neuromas with increasing frequency. The cases of amputation neuromas found between 1950 and 1955 were reviewed to determine whether anything could be learned of their relationship to the patients' symptoms or their possible influence on the function of the biliary tract, or both. History The first reported neuroma of the biliary tract, reported by Husseinoff 6 in 1928, was located at the cystic duct stump. Similar findings were described by Shapiro and Lifvendahl 8 in 1931. In both instances the neuromas were postmortem findings. The first neuroma of the bile ducts described in a clinical patient was reported by Comfort and Walters 4 in 1931. It was associated with biliary obstruction. The next report was published by Cieslak and Stout 3 in 1946; they

Background Hepatocellular carcinoma (HCC) with biliary tumor thrombus (BTT) is rare and its impact on postoperative prognosis remains controversial. The aim of this study was to evaluate the published evidence concerning the outcome of surgical resection of HCC with BTT. Methods Eligible studies were identified by searching PubMed and reviewed systematically. Comparisons of the clinicopathologic features and surgical outcomes for HCC patients with or without BTT were analyzed using meta-analytical techniques. Results Twenty retrospective studies containing 598 patients that met the selection criteria were included for review. The perioperative mortality was 2.1 % (range, 0–10 %), and the median 5-year overall survival (OS) was 24 % (range, 0–48 %) with a recurrence rate of 63.9 % (range, 42–91 %). Pooled analysis of 13 comparative studies showed that HCC patients with BTT had a higher incidence of vascular invasion (odds ratio [OR]: 4.70, 95 % CI: 2.90–7.60; P

The UK incidence of pancreatic ductal adenocarcinoma (PDAC) is approximately 9/100,000 population compared with 1-2/100,000 for biliary tract cancer (BTC). This study explores the incidence of these cancers over time and the influence of socio-demographic and geographic factors in a UK primary care cohort.

Obesity and associated metabolic disorders have been implicated in liver carcinogenesis; however there is little data on the role of obesity-related biomarkers on liver cancer risk. We studied pros ...

Background: There is increasing evidence suggesting that shift work involving night work may increase cancer risk. Methods: We examined the association between working rotating shifts and the risk of death from biliary tract cancer among Japanese men who participated in the Japan Collaborative Cohort Study. Of the 46, 395 men recruited, 22, 224 men aged 40-65 at baseline (1988-1990) who reported working full-time or were self-employed were included in the present analysis. The study subjects were followed through December 31, 2009. Information regarding occupation and lifestyle factors was collected using a self-administered questionnaire. Cox proportional hazard models were used to estimate the hazard ratio (HR) and 95 % confidence interval (CI) for the risk of death from biliary tract cancer in relation to shift work. Results: During a mean 17-year follow-up, we observed 94 biliary tract cancer deaths, including 23 deaths from gallbladder cancer and 71 deaths from extrahepatic bile duct cancer. Overall, shift work was associated with a statistically non-significant increase in the risk of biliary tract cancer, with an HR of 1.50 (95 % CI: 0.81-2.77), among rotating shift workers. When the analysis was limited to extrahepatic bile duct cancer, a significant association appeared, with a multivariable-adjusted HR of 1.93 (95 % CI: 1.00-3.72) for rotating shift workers. Conclusion: Our data indicate that shift work may be associated with increased risk of death from extrahepatic bile duct cancer in this cohort of Japanese men. The association with gallbladder cancer remains unclear because of the small number of deaths.

Aims Advanced biliary tract carcinomas (ABC) are malignancies with limited effective therapies for advanced disease. There is little published evidence of outcomes of ABC patients participating in phase I clinical trials. Materials and methods Patient characteristics, treatment details and outcomes of ABC patients treated at a dedicated phase I unit were captured and analysed from case and trial records. Results In total, 123 ABC patients were included in the study, of which 48 patients participated in 41 different phase I trials; 75 (61%) did not participate due to rapid disease progression or patient choice. Molecular characterisation of tumours using a targeted panel was conducted in 15 (31%), yielding several potentially actionable mutations, including BRCA , PIK3CA , FGFR , AKT and PTEN loss. Of the 39 evaluable patients there was one exceptional responder. Eighteen (46%) other patients achieved stable disease as their best response, with a clinical benefit rate at 4 months of 10%. Treatment was generally well tolerated with grade 3 or 4 adverse events only observed in eight patients (17 %), of which six were drug related and led to trial discontinuation in one (3%), with no toxicity-related deaths. Conclusion Carefully selected ABC patients have been found to tolerate experimental phase I clinical trials without excess toxicity. The aggressive nature of this disease warrants consideration of early referral to a phase I unit. Future work will require comprehensive molecular profiling in an attempt to understand the biology underlying the exceptional responders and to match patients in real-time to targeted therapies.

BACKGROUND: Percutaneous stent placement is an accepted method of palliation in malignant biliary obstruction. Factors predicting early mortality after this procedure have not been identified. METHODS: We performed a retrospective study of 141 patients with malignant biliary obstruction who underwent percutaneous stent placement for biliary decompression to identify the risk factors associated with early mortality (< or = 30 days). RESULTS: Of 14 clinicopathological and laboratory variables analysed blood urea, albumin, haemoglobin and alkaline phosphatase were found to be significant on univariate analysis. The age and gender of the patient along with cancer type, level of obstruction, presence of pyrexia and bilirubin level had no influence on early mortality. Stepwise logistic regression identified the haemoglobin level and blood urea to be independently significant in predicting early mortality. Overall 30-day mortality was 20.5% (29/141). Patients with blood urea over 4.3 mmol/l and a haemoglobin less than 10.9 g/dl had a mortality rate of 52% (12/23) compared with 14% (17/118) in the remainder. Using these two variables a regression equation has been derived which allows calculation of the probability of survival at 30 days after the percutaneous procedure. CONCLUSIONS: Laboratory variables in patients with malignant obstructive jaundice can be used to predict mortality following percutaneous stent insertion

The aim of the study was to assess associations between intake of combined soft drinks (sugar sweetened and artificially sweetened) and fruit and vegetable juices and the risk of hepatocellular carcinoma (HCC), intrahepatic bile duct (IHBC) and biliary tract cancers (GBTC) using data from the European Prospective Investigation into Cancer and Nutrition cohort of 477,206 participants from 10 European countries. After 11.4 years of follow-up, 191 HCC, 66 IHBC and 236 GBTC cases were identified. Hazard ratios and 95 % confidence intervals (HR; 95 % CI) were estimated with Cox regression models with multivariable adjustment (baseline total energy intake, alcohol consumption and intake pattern, body mass index, physical activity, level of educational attainment and self-reported diabetes status). No risk associations were observed for IHBC or GBTC. Combined soft drinks consumption of >6 servings/week was positively associated with HCC risk: HR 1.83; 95 % CI 1.11–3.02, p trend = 0.01 versus non-consumers. In sub-group analyses available for 91 % of the cohort artificially sweetened soft drinks increased HCC risk by 6 % per 1 serving increment (HR 1.06, 95 % CI 1.03–1.09, n cases = 101); for sugar-sweetened soft drinks, this association was null (HR 1.00, 95 % CI 0.95–1.06; n cases = 127, p heterogeneity = 0.07). Juice consumption was not associated with HCC risk, except at very low intakes (

The patient described in this report represents the first reported case of Trousseau's syndrome caused by a malignancy arising in a choledochal cyst. The clinical features were classic, with thrombophlebitis migrans, resistance to anticoagulation, and an occult, mucin-producing adenocarcinoma. The increased recognition of the malignant potential of choledochal cysts should encourage clinicians to consider these unusual anomalies in patients with jaundice or other abnormalities of hepatic function. When possible, the cyst should by excised to minimize the risk of malignant degeneration.

A case of adenocarcinoma of the colon with a secondary lesion in the lumen of the common bile duct shown by transhepatic cholangiography is described. The differential diagnosis includes stones, blood clot, and benign and malignant tumors.

Percutaneous transhepatic biliary drainage is usually used for preoperative decompression and for palliation in obstructive jaundice, but little attention has been focused on catheter complications. We retrospectively reviewed our experience with percutaneous transhepatic biliary drainage in 81 consecutive patients. There was a 10.0% failure rate and an 8.6% mortality rate. Four patients (4.9%) required emergency operations for complications and an additional ten patients (12.3%) required transfusions. The overall sepsis rate was 34.6%; prophylactic antibiotics decreased the sepsis rate. Thirty-eight patients (47.0%) required 68 manipulation procedures for catheter malfunctions. No distinguishing characteristics could be found to identify the subgroups having complications or mortalities. The efficacy of preoperative percutaneous transhepatic biliary drainage has not been proved, and the indications for palliation are not clearly defined. Further trials are needed to define the role of percutaneous transhepatic biliary drainage in patients with obstructive jaundice. (JAMA1986;255:2763-2767)

Benign tumors of the biliary passages are rare and are seldom considered in the differential diagnosis of obstructive jaundice. The number of recorded cases is increasing, and it is apparent from the case histories that failure to discover the tumor may result in disaster. Recently, at the New York Hospital, we have had opportunity to observe and to operate on a patient with obstructive jaundice caused by a large papilloma arising in the left hepatic duct and obstructing the common bile duct. This case stimulated us to review the literature and is the subject of this report. Benign intrahepatic tumors, apparently originating from the smaller biliary radicals, are commoner than lesions of the main biliary passages. According to Ewing, 1 cysts may arise within cirrhotic livers from obstruction of the ducts and are usually multiple and small. Not infrequently, congenital cysts of the liver are associated with polycystic disease of

Background The pathogenesis of biliary cancers is ill-defined. This study investigates changes in gene expression and copy number in biliary cancers and correlates these changes with anatomical site of origin, histopathology and outcome. Methods We performed gene expression and CGH analysis on 34 biliary tract cancer specimens. Results were confirmed by RT-PCR. Clinical-pathologic correlation was made using functional over-representation analysis of the top 100 mutations associated with each variable. Results There were 545 genes with altered expression in extrahepatic cholangiocarcinoma, 2,354 in intrahepatic cholangiocarcinoma, and 1,281 in gallbladder cancer. Unsupervised hierarchical clustering analysis indicated there was no difference in the global gene expression patterns between each biliary cancer subgroup. CGH analysis revealed that short segments of chromosomes 1p, 3p, 6q, 8p, 9p, and 14q were commonly deleted across all cancer subtypes. Commonly amplified regions included segments of 1q, 3q, 5p, 7p, 7q, 8q, and 20q. Over-representation analysis revealed an association between altered expression of functional gene groupings and pathologic features. Conclusion This study defined regions of the genome associated with changes in DNA copy number and gene expression in specific subtypes of biliary cancers. The findings have implications for identification of therapeutic targets, screening, and prognostication.