(See also Overview of Biliary Function.)
Cholangiocarcinomas and other bile duct tumors are rare (1 to 2/100,000 people) but are usually malignant. Cholangiocarcinomas occur predominantly in the extrahepatic bile ducts: 60 to 70% in the perihilar region (Klatskin tumors), about 25% in the distal ducts, and the rest in the liver. Risk factors include primary sclerosing cholangitis, older age, infestation with liver flukes, and a choledochal cyst.
Gallbladder carcinoma is uncommon (2.5/100,000). It is more common among American Indians, patients with large gallstones (> 3 cm), and those with extensive gallbladder calcification due to chronic cholecystitis (porcelain gallbladder). Nearly all (70 to 90%) patients also have gallstones. Median survival is 3 months. Cure is possible when cancer is found early (eg, incidentally at cholecystectomy).
Gallbladder polyps are usually asymptomatic benign mucosal projections that develop in the lumen of the gallbladder. Most are < 10 mm in diameter and composed of cholesterol ester and triglycerides; the presence of such polyps is called cholesterolosis. They are found in about 5% of people during ultrasonography. Other, much less common benign polyps include adenomas (causing adenomyomatosis) and inflammatory polyps. Small gallbladder polyps are incidental findings that do not require treatment.
Most patients with cholangiocarcinomas present with pruritus and painless obstructive jaundice, typically at age 50 to 70 years. Early perihilar tumors may cause only vague abdominal pain, anorexia, and weight loss. Other features include fatigue, acholic stool, a palpable mass, hepatomegaly, or a distended gallbladder (Courvoisier sign, with distal cholangiocarcinoma). Pain may resemble that of biliary colic (reflecting biliary obstruction) or may be constant and progressive. Sepsis (secondary to acute cholangitis), although unusual, may be induced by endoscopic retrograde cholangiopathy (ERCP).
Manifestations of gallbladder carcinoma may range from incidental findings at cholecystectomy done to relieve biliary pain to cholelithiasis to advanced disease with constant pain, weight loss, and an abdominal mass or obstructive jaundice.
Most gallbladder polyps cause no symptoms.
Ultrasonography (sometimes endoscopic), followed by CT cholangiography or magnetic resonance cholangiopancreatography (MRCP)
Sometimes endoscopic retrograde cholangiopancreatography (ERCP)
Cholangiocarcinomas and gallbladder carcinomas are suspected when extrahepatic biliary obstruction is unexplained. Laboratory test results reflect the degree of cholestasis. In patients with primary sclerosing cholangitis, serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) levels 19-9 are measured periodically to check for cholangiocarcinoma.
Diagnosis is based on ultrasonography (or endoscopic ultrasonography), typically followed by MRCP (see Imaging Tests of the Liver and Gallbladder). CT is sometimes done and may provide more information than ultrasonography, particularly for gallbladder carcinomas. When these methods are inconclusive or if cholangiocarcinoma is suspected, endoscopic retrograde cholangiopancreatography (ERCP) is necessary. ERCP not only detects the tumor but also, with brushings, can provide a tissue diagnosis, sometimes making ultrasonography- or CT-guided needle biopsy unnecessary. Contrast-enhanced CT assists in staging.
Open laparotomy is necessary to determine disease extent, which guides treatment.
For cholangiocarcinoma, stenting or surgically bypassing the obstruction relieves pruritus, jaundice, and perhaps fatigue.
Hilar cholangiocarcinomas with CT evidence of spread are stented via percutaneous transhepatic cholangiography or endoscopic retrograde cholangiopancreatography (ERCP). Distal duct cholangiocarcinomas are stented endoscopically with ERCP. If cholangiocarcinoma appears localized, surgical exploration determines resectability by hilar resection or pancreaticoduodenectomy.
Liver transplantation for localized hilar cholangiocarcinoma is available at some transplant centers as part of a specific protocol approved by the United Network for Organ Sharing (UNOS).
Many gallbladder carcinomas are treated symptomatically.
Biliary tract cancer (usually cholangiocarcinoma or gallbladder carcinoma) is uncommon.
Suspect cancer if patients have an unexplained extrahepatic biliary obstruction or abdominal mass.
Diagnose cancers by imaging, beginning with ultrasonography, followed by MRCP.
Treat cancers symptomatically (eg, by stenting or bypassing obstructions in cholangiocarcinoma); occasionally, resection is warranted.
Consider liver transplantation for selected patients with hilar cholangiocarcinoma.