ABSTRACT

Hilar cholangiocarcinoma remains a challenging disease for both doctor and patient. Of all tumors arising in the biliary tree it is the most difficult to manage. The majority of patients will die within 6 months to a year of diagnosis if not treated; death is usually related to local tumor spread and the effects of biliary obstruction and cholangitis leading to liver failure.1,2

The purpose of this chapter is to define a systematic approach to diagnosis, work-up and treatment of hilar cholangiocarcinoma based on the following premises:

1. Preoperative diagnosis of cholangiocarcinoma is almost always possible without the need for tissue diagnosis. The possibility of a benign localized stricture mimicking a malignant one has to be considered.3