Cholangiocarcinoma (CCA) is a lethal cancer of the biliary epithelium, originating from the liver (intrahepatic), at the confluence of the right and left hepatic ducts (hilar) or in the extrahepatic bile ducts. It is a rare malignancy associated with poor prognosis.

We searched the PubMed/MEDLINE database for relevant articles published from 1989 to 2008. The search terms used were related to "cholangiocarcinoma" and its "treatment". Although no language restrictions were imposed initially, for the full-text review and final analysis, our resources only permitted the review of articles published in English. This review deals with the treatment of cholangiocarcinoma, the principles and the current trends.

The risks and prognostic factors, symptoms and differential diagnosis are thoroughly discussed. In addition, the tools of preoperative diagnosis such as endoscopic retrograde cholangiopancreatography, digital image analysis, fluorescence in situ hybridization and magnetic resonance cholangiopancreatography are reviewed. Moreover, the treatment of CCA is discussed.

The only curative treatment available is surgical management. Unfortunately, many patients present with unresectable tumors, the majority of whom die within a year of diagnosis. Surgical treatment involves major resections of the liver, pancreas and bile duct, with considerable mortality and morbidity. However, in selected cases and where indicated, appropriate management with aggressive surgery may achieve a good outcome with a prolonged survival expectancy.

Although the role of intraductal ultrasound (IDUS) in the evaluation of specific disease entities is well known, its utility in evaluating indeterminate findings in a heterogeneous group of patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) is unknown. This study evaluates the diagnostic accuracy of IDUS in patients with indeterminate findings at ERCP.

This was a prospective study of all patients who underwent IDUS for evaluation of an indeterminate biliary stricture or main pancreatic duct (MPD) dilation noted at ERCP over an 8-month period. The accuracy of IDUS was established based on long-term follow-up, surgery, or further investigations.

Twenty-nine (5%) of 600 patients who underwent ERCP had an indeterminate finding that warranted further evaluation by IDUS: this was biliary stricture in 19 patients and MPD dilation in 10. Technical success was 100%. Mean duration of follow-up was 435 days (range 192-614 days). In patients with biliary stricture, IDUS diagnosed 11 as benign and eight as malignant. In patients with MPD dilation, IDUS diagnosed intraductal papillary mucinous tumor in six patients and chronic pancreatitis in four. Findings on IDUS supported the correct diagnosis in 27 of 29 patients (93%). In two patients with dominant hilar stricture in the setting of primary sclerosing cholangitis, IDUS was false positive in one and false negative in the other. One patient died of multiorgan failure due to post-ERCP pancreatitis.

A technically easy procedure, IDUS offers unique advantages in the evaluation of patients with indeterminate findings at ERCP.

Hilar tumors have proven to be a challenge to treat and manage because of their poor sensitivity to conventional therapies and our inability to prevent or to detect early tumor formation. Endoscopic stent drainage has been proposed as an alternative to biliary-enteric bypass surgery and percutaneous drainage to palliate malignant biliary obstruction. Prosthetic palliation of patients with malignant hilar stenoses poses particular difficulties, especially in advanced lesions (type II lesions or higher). The risk of cholangitis after contrast injection into the biliary tree in cases where incomplete drainage is achieved is well known. The success rate of plastic stent insertion is around 80% in patients with proximal tumors. Relief of symptoms can be achieved in nearly all patients successfully stented.

Black oesophagus is a rare pathological condition of unknown aetiology characterised by a full length, circumferential black discolouration of the entire oesophageal mucosa. The disease is sporadically encountered during upper gastrointestinal endoscopy. We used conventional histology, enzyme histochemistry and immunohistology to examine five autopsy cases (four males, one female; age range 43-86 years) of black oesophagus. On microscopical examination, the esophageal mucosa was entirely necrotic with demarcation by a leukocytic infiltrate in the upper submucosa. This infiltrate was dominated by neutrophilic granulocytes and scattered macrophages lacking haemosiderin deposits, placing the noxious mucosal injury in a time frame of approximately 24-72 h prior to death. Black oesophagus was established as the immediate cause of death in one case due to desanguination from the oesophagus and significantly contributed to the fatal outcome in a second case. Apart from a history of chronic alcohol consumption in four cases, no other pre-existing pathological or debilitating conditions could be established. The remarkably consistent pathomorphological picture of the disease seems to be the result of impaired microcirculation of the oesophageal mucosa due to prolonged hypotension of variable aetiology. The diagnosis of black oesophagus requires exclusion of other causes such as ingestion of caustic materials and should be based on histological examination.

Endoscopic treatment of pancreatobiliary malignancies has been recognized in the last decades as the treatment of choice in inoperable patients. Endoscopic armamentarium includes biliary stents (plastic and metallic) to bypass neoplastic strictures of the biliary tree, and pancreatic stents to palliate the obstructive pain caused by stenoses of the main pancreatic duct. A major issue is the long-term patency of plastic stents that will eventually clog on average after 3 to 4 months. Self-expandable metallic stents have longer patency, but they can also become occluded by tumor ingrowth or overgrowth; they are also much more expensive; their use is thus recommended in patients with longer life expectancy. Decompression of the dilated main pancreatic duct in pancreatic carcinoma may be effective in the relief of obstructive pain. Endoscopic palliation in pancreatic and biliary malignancies appears safe and effective; management of patient in referral centers, with an available team of gastroenterologists with endoscopic skills, surgeons, and radiologists is recommended.