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Pathogenesis And Epidemiology

Cancer of the bile duct, cholangiocarcinoma, can occur anywhere along the biliary tract from the ampulla of Vater to the intrahepatic biliary radicals. The hepatic duct bifurcation is the most frequently involved site accounting for approximately 60–80% of cholangiocarcinomas.

The etiology of cholangiocarcinoma has not been clearly defined. A number of pathologic conditions, however, resulting in either acute or chronic biliary tract epithelial injury may predispose to malignant change. Primary sclerosing cholangitis, an idiopathic inflammatory condition of the biliary tree, has been clearly associated with the development of cholangiocarcinoma in up to 40% of patients. Congenital biliary cystic disease, such as choledochal cysts or Caroli's disease, has also been associated with malignant transformation in up to 25% of cases. These conditions appear to be related to an anomalous pancreatico-biliary duct junction and, perhaps, are related to the reflux of pancreatic secretions into the bile duct.

Take Home Points

Cholangiocarcinoma is a rare disease but has an increased incidence in patients with underlying sclerosing cholangitis or choledochal cysts.

Perihilar cholangiocarcinoma, also called Klatskin's tumor, is the most common. Klatskin's tumors are classified with the Bismuth-Corlette system.

Surgical resection is the only curative treatment for cholangiocarcinoma. Resectability is largely determined by the extent of ductal and vascular involvement.

Patients have a better than 5-year survival rate when an R0 resection is obtained.

Diagnosis And Staging

Jaundice is a presenting complaint in almost all cases of Klatskin tumors. Non-specific complaints, such as weight loss and abdominal pain, may also occur. Cholangitis is an uncommon symptom at the time of presentation of cholangiocarcinoma, but frequently occurs after endoscopic or percutaneous biliary tract instrumentation.

The evaluation of the jaundiced patient should begin with either an abdominal ultrasound or computed tomography (CT) scan. Findings on either study suggestive of Klatskin tumor include a dilated intrahepatic biliary tree, a normal or collapsed gallbladder and extrahepatic biliary tree, and a normal pancreas. Contrast enhanced spiral CT scanning provides highly accurate technique for imaging small lesions of biliary tree and clarifying the relationship of the tumor and portal venous and arterial structures. More recently, the use of magnetic resonance cholangiopancreatography (MRCP) has been able to clearly identify the site and nature of biliary obstruction in a noninvasive manner.

After biliary ductal demonstration has been demonstrated by imaging studies, it is imperative for proximal biliary tumors that cholangiography be performed. Cholangiography should define not only the location, but also visualize the uppermost extent of the tumor to determine the limits of resectability.

Surgical Treatment

At completion of preoperative staging, approximately two-thirds of patients with Klatskin tumors will be considered operable. Resectability is further assessed at operation by careful exploration for peritoneal implants, hepatic metastasis and regional lymph node involvement. The goal of any surgical procedure is to remove all gross and microscopic disease and to relieve biliary obstruction. The standard surgical procedure includes resection of the extrahepatic bile duct from the duodenum to above the level of the hepatic confluence, cholecystectomy, portal lymphadenectomy and biliary-enteric anastomosis. Controversy, however, exists with respect to the performance of routine, hepatic lobectomy and caudate lobectomy. Unfortunately, there have been no prospective, randomized trials to address this controversy.

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