EPIDEMIOLOGY

Intrahepatic cholangiocarcinoma (ICC) is primary liver cancer with cholangiocytic molecular and histopathologic characteristics located peripheral to the biliary ductal confluence. Cholangiocarcinomas, in general, represent only 15% of primary liver cancers (1), with ICC composing only about 15% of those. These numbers have contributed to the pervasive perception that ICC is a rare cancer, and compared with cancers that globally impact health care (i.e., colon, breast, lung cancers), indeed it is. However, an accelerated increase in incidence has recently been demonstrated, which, coupled with the very poor prognosis imparted by this cancer, has raised concerns. From 1975 to 2000, the age-adjusted incidence of ICC in the United States has increased from 0.32 per 100,000 to 0.85 per 100,000, or roughly an increase from 800 to 2800 newly diagnosed cases per year (2). More concerning is that this 165% increase in age-adjusted incidence (2, 3) is accelerating, with current estimates showing an annual percentage increase in incidence of 9.11% (2). Prognosis is almost universally poor, with little change in demonstrated survival over the past 25 years despite novel and more aggressive treatments. In 1975, the 1- and 5-year survival rates were 15.8% and 2.6% respectively. Twenty-five years later (2000) and despite significant medical advances in cancer treatment 1-, 2- and 5-year survival rates have essentially remained stable, at 25%, 13% and 3.5% respectively (2, 3). Even for the few patients who are initially deemed resectable, post-operative 1-, 3- and 5-year survival is 58%, 33% and 33%, respectively (4, 5).