Multidisciplinary management of perihilar bile duct cancer.

Abstract

Cancers of the extrahepatic biliary system remain a challenge to surgical, medical, and radiation oncologists. Although surgical resection is the only curative option, many patients are incurable at the time of diagnosis and palliative treatment is often undertaken. Understanding the options for surgical resection and the limits of operative intervention is critical in caring for these patients. Cholangiocarcinoma may occur intrahepatically, or extrahepatically in perihilar or distal ductal locations. Perihilar lesions constitute approximately 75% of tumors. Distal bile duct tumors and intrahepatic tumors follow in frequency. This anatomic classification has therapeutic relevance. Intrahepatic tumors are treated with liver resection; distal tumors are managed, as are pancreatic cancers, with pancreaticoduodenectomy. Perihilar cancers require a combination of operative and nonoperative approaches and will be the focus of the current discussion. In the United States strong associations have been made between cholangiocarcinoma and biliary cystic disease, sclerosing cholangitis, and ulcerative colitis. In autopsy series as many as 40% of patients with sclerosing cholangitis have been shown to have cholangiocarcinoma, often undiagnosed in life. Approximately one third of patients undergoing hepatic transplantation for liver failure secondary to sclerosing cholangitis are discovered, on histologic examination of the recipient hepatectomy, to have bile duct cancer. The association of sclerosing cholangitis with ulcerative colitis provides the link of the latter condition with bile duct cancer. In East Asia, liver flukes and hepatolithiasis are both associated with cholangiocarcinoma. The large majority (90%) of bile duct tumors are adenocarcinomas. Most lesions demonstrate a diffuse infiltrative pattern of growth with a strong desmoplastic component, making the pathologic demonstration of malignancy notoriously difficult. Percutaneous fineneedle aspiration biopsy has the highest diagnostic yield, although biopsy is often hampered by the absence of perihilar mass effect. Luminal brush biopsy and cytologic examination of bile are alternatives of lower diagnostic value. The typical patient is between 60 and 65 years of age; men and women are affected with approximately equal frequency. Jaundice will develop in greater than 90% of patients with perihilar cholangiocarcinoma. Serum liver tests demonstrate hyperbilirubinemia and disproportionate elevation in alkaline phosphatase. Most patients will have a serum bilirubin level greater than 10 mg/dL. Bilirubin elevations to this level are unusual for nonmalignant causes of jaundice. Serum levels of carcinoembryonic antigen (CEA), -fetoprotein, and CA 19-9 are usually normal when biliary obstruction is relieved. Cholangitis is not usually a presenting problem in the absence of earlier biliary manipulation. Anorexia, upper abdominal pain, and weight loss are common, but nonspecific, symptoms.