Hilar cholangiocarcinoma is an uncommon cause of malignant biliary obstruct
ion marked by local tumor spread for which surgery offers the only chance o
f cure. The diagnostic evaluation and surgical management of this disease c
ontinues to evolve. Although direct cholangiography and endoscopic biliary
procedures have been used extensively to anatomically define the extent of
tumor involvement, establish biliary decompression, and obtain histological
confirmation of tumor, reli ance on these invasive procedures is no longer
necessary, and may be detrimental. Current noninvasive imaging technology
permits accurate staging of the primary tumor and has improved patient sele
ction for operative intervention without the need for invasive procedures.
Overall survival has improved in accordance with an increasingly aggressive
surgical approach. The propensity of this tumor for local invasion has led
most experienced hepatobiliary centers to perform a partial hepatectomy in
50% to 100% of cases. Three-year survival rates of 35% to 50% can be achie
ved when negative histological margins are attained at the time of surgery.
When resection is not feasible, either operative bilioenteric bypass or pe
rcutaneous transhepatic intubation can achieve significant palliation, Ther
e is no effective adjuvant therapy for this disease, and unless clear indic
ations of unresectability exist, most patients should be considered for sur
gical exploration.