Extrahepatic Bile Duct Tumors

Benign Tumors

Benign tumors of the bile ducts are rare; papilloma and adenoma are the most common lesions. Symptoms include intermittent jaundice and right upper quadrant pain. The treatment of choice is local excision.

Malignant Tumors

By far the most common malignant tumor is adenocarcinoma. Bile duct cancer is more common in men; the average age at diagnosis is 60. Tumors of the upper portion of the ducts (50% of all lesions) are intimately related to the liver; those of the middle portion, to the portal vein and hepatic artery; and those of the lower portion, to the pancreas and duodenum.

Predisposing factors include primary sclerosing cholangitis, Opisthorchis (Clonorchis) sinensis infestation, and industrial exposure (in automobile and rubber manufacturing plant workers).

Symptoms, Signs, and Diagnosis

Because of their location, these tumors usually cause symptoms early, with jaundice occurring in almost all patients. Right upper quadrant pain occurs in > 50% of cases. Other symptoms and signs include weight loss, nausea, vomiting, anorexia, fever, chills, and hepatomegaly. If obstruction occurs below the cystic duct, the gallbladder may be palpable (Courvoisier's sign).

Diagnosis is made by ultrasound and CT. Percutaneous transhepatic cholangiography and endoscopic retrograde cholangiopancreatography provide more accurate localization of the tumor. Liver function test results are consistent with extrahepatic obstruction.

Prognosis and Treatment

The 5-year survival rate is only 5%. Tumors in the proximal portion of the bile duct system rarely are operable. Resectable tumors of the central portion of the bile ducts may be treated by local en bloc excision. For resectable tumors of the distal portion, radical resection and pancreatoduodenectomy (Whipple's operation) provide some promising benefit with 5-year survival rates of 20 to 30%, but patients > 70 have a high operative risk.

For the majority of patients with advanced extrahepatic biliary carcinoma, no effective treatment exists. Palliation may be accomplished by dilation and stent insertion either endoscopically or via transhepatic cholangiography perhaps combined with intrabiliary radiation. Death and dying issues must be addressed.