Hilar cholangiocarcinoma: a review and commentary.

Hilar cholangiocarcinoma is an uncommon cause of malignant biliary obstruction marked by local tumor spread for which surgery offers the only chance of cure. The diagnostic evaluation and surgical management of this disease continues 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, reliance 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 selection 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 achieved when negative histological margins are attained at the time of surgery. When resection is not feasible, either operative bilioenteric bypass or percutaneous transhepatic intubation can achieve significant palliation. There is no effective adjuvant therapy for this disease, and unless clear indications of unresectability exist, most patients should be considered for surgical exploration.