Primary neoplasms of the extrahepatic biliary tract are found at postmortem examination at a rate less than 0.5 per cent. Discussions of bile duct tumours generally exclude carcinomas isolated to the intrahepatic ducts, gallbladder, pancreas, and ampulla of Vater. Bile duct cancer is more common in men (male : female ratio of approximately 1.5 : 1), with a peak incidence in the seventh decade of life.

The site of the cancer is the most important prognostic factor in bile duct cancer. Fifty to 75 per cent of cancers are located in the upper third of the extrahepatic biliary tract at the level of the common hepatic duct or hepatic duct bifurcation. Cancers of the distal common bile duct or cystic duct–common duct junction are less common. An uncommon diffuse form of bile duct cancer carries a particularly poor prognosis.

Bile duct tumours are typically small, firm, well-circumscribed lesions involving the full thickness of the bile duct wall; they may occasionally have polypoid projections into the lumen of the duct. These tumours are adenocarcinomas with varying degrees of differentiation. The incidence of local metastases at operation approaches 50 per cent, spread to the liver and hepatoduodenal ligament being most common. Local spread does not necessarily represent a contraindication to resection. Metastases to distant organs are uncommon at the time the cancer is identified.

Jaundice is the most common presenting symptom among patients with bile duct cancer, followed by weight loss, abdominal pain, and pruritus. Less frequently reported are signs and symptoms of cholangitis. Serum liver function tests typically reveal an obstructive pattern, often with marked elevations in total and conjugated bilirubin levels. As its clinical presentation is similar to that of benign biliary tract abnormalities, bile duct cancer is often unrecognized. Delays in diagnosis of several months are typical; many patients undergo several non-therapeutic procedures prior to the establishment of a definitive diagnosis.

Although radiographic imaging of bile duct tumours usually starts with ultrasonography or CT, detailed anatomical information regarding the site and the extent of the obstructive lesion is best obtained by endoscopic retrograde cholangiopancreatography or transhepatic cholangiography. When the diagnosis is made intraoperatively, operative cholangiography and choledochoscopy are helpful. Intraoperative choledochoscopy should be performed as a routine preliminary step to a planned curative procedure to exclude unsuspected multicentricity.

The mainstay of treatment for patients with carcinoma of the extrahepatic bile ducts is surgery. The types of procedures performed fall into three categories: potentially curative resection, palliative surgical bypass, and operative intubation and drainage of ductal obstruction. Drainage can also be performed non-operatively.

Resection of the tumour generally entails anastomosis of the proximal ductal system to a loop of small intestine. The specific procedure selected depends upon the location and extent of the lesion. Approximately two-thirds of middle and distal common duct tumours are resectable, compared with less than one-third of proximal lesions. Recent advances in selection of patients and in perioperative care have led to marked reductions in operative mortality associated with tumour resection: operative mortality rates are now less than 5 per cent, even in elderly patients. The relative safety of these major biliary resections has led some authors to advocate radical vascular and hepatic resections for locally invasive proximal lesions. The impact of this aggressive approach on long-term survival is uncertain.

Palliative procedures aim to relieve biliary obstruction and its attendant sequelae. Most patients undergo operative palliation by either bilioenteric bypass of the obstructed segment(s) or ductal intubation. The advantages of operative versus non-operative palliation for the patient who is a candidate for surgery include accurate staging, pathological confirmation of the diagnosis, and possibly, improved biliary drainage and quality of life.

Both radiation and chemotherapy are employed as adjuvant treatments in patients with bile duct cancer. These treatments are generally delivered postoperatively for residual local disease. Prospective randomized studies evaluating the potential benefit of these adjuvant therapies are unavailable.

Overall survival figures for patients with bile duct cancer remain disappointing: less than half survive 1 year and less than 10 per cent are alive at 5 years. Patients with proximal cancers have a particularly poor prognosis. Most series report mean survival times of less than 1 year, and 5-year survival is extremely rare. Clearly, patients with neoplasms that are suitable for resection have the best prognosis.

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