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Gallbladder and bile duct tumors can cause extrahepatic biliary obstruction. Symptoms may be absent but often are constitutional or reflect biliary obstruction. Diagnosis is based on ultrasonography plus CT cholangiography or magnetic resonance cholangiopancreatography. Prognosis is grim. Mechanical bile drainage can often relieve pruritus, recurrent sepsis, and pain due to biliary obstruction.
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 mo. 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.
Symptoms and Signs
Most patients with cholangiocarcinomas present with pruritus and painless obstructive jaundice, typically at age 50 to 70 yr. Early perihilar tumors may cause only vague abdominal pain, anorexia, and weight loss. Other features include acholic stool, a palpable mass, hepatomegaly, or a distended gallbladder (Courvoisier's sign, with distal cholangiocarcinoma). Pain may resemble that of biliary colic (reflecting biliary obstruction) or may be constant and progressive. Sepsis (acute cholangitis), though unusual, may be induced by ERCP.
Manifestations of gallbladder carcinoma may range from incidental findings at cholecystectomy done for 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.
Diagnosis
Cholangiocarcinomas 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 cancer antigen (CA) levels 19-9 are used for surveillance to detect the development of cholangiocarcinoma. Diagnosis is based on ultrasonography (or endoscopic ultrasonography) and CT cholangiography or magnetic resonance cholangiopancreatography. When these methods are inconclusive, ERCP with percutaneous transhepatic cholangiography (PTC) becomes 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.
Gallbladder carcinomas are better defined by CT than by ultrasonography. Open laparotomy is necessary to determine disease extent, which guides treatment.
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 PTC or ERCP. Distal duct cholangiocarcinomas are stented endoscopically with ERCP. If cholangiocarcinoma appears localized, surgical exploration determines resectability by hilar resection or pancreaticoduodenectomy. However, successful resection is uncommon.
Liver transplantation is not indicated because of the high recurrence rate. Effectiveness of adjuvant chemotherapy and radiation therapy for cholangiocarcinomas is unproved as yet.
Many gallbladder carcinomas are treated symptomatically.
Last full review/revision December 2007 by Eldon A. Shaffer, MD
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