ABSTRACT

Cancers arising from the gallbladder or biliary epithelium account for approximately 15% of hepatobiliary neoplasms. This amounts to approximately 7500 new diagnoses per year in the United States. Gallbladder cancer is the most common site, accounting for 60% of the biliary tract cancer. The remaining 40% are referred to as cholangiocarcinomas and are distributed throughout the extrahepatic and intrahepatic biliary tree (1). Coupling the uncommon incidence of these tumors with their commonly advanced stage at presentation greatly hampers efforts to conduct meaningful randomized clinical trials. Complete resection is associated with the best survival and is the most effective therapy but is usually only possible in a minority of patients. Palliating the effects of biliary obstruction is thus often the primary therapeutic goal. Chemotherapy and radiation therapy have not been proven to reduce the incidence of recurrence after resection nor to improve survival. In addition, only a few chemotherapeutic agents have demonstrated marginal activity in patients with unresectable disease. It must be emphasized that meaningful controlled data comparing different treatment modalities are largely nonexistent. This chapter focuses on primary malignancies of the biliary tree, with an emphasis on current treatment paradigms for the most common of these tumors: hilar cholangiocarcinoma and gallbladder carcinoma.