Intrahepatic cholangiocarcinoma. Current management.

Peripheral/Intrahepatic Cholangiocarcinoma (IHC), a malignant epithelial tumor originating from the intrahepatic bile ducts, is the second most common primary liver cancer after hepatocellular carcinoma. Unlike hepatocellular carcinoma, however, IHC is infrequently associated with chronic underlying liver disease. A recent study from the United States has demonstrated a 9% annual percentage increase and an overall 10-fold increase in mortality related to IHC since 1973. Despite this observation, however, IHC remains a rare disease in Western countries. Of the approximately 4000 patients seen at Memorial Sloan-Kettering Cancer Center with hepatic lesions since from 1995-2001, 7% had a diagnosis of cholangiocarcinoma and only 1% had IHC. As a result, progress in elucidating the pathogenesis and clinical behavior of these tumors has been slow. However, the increasing number of reports in recent years has increased awareness of IHC and provided some insight into its biology. At present, complete resection is the only therapy that offers the possibility of long-term survival. Single agent or combination chemotherapy and radiation therapy have not been shown to have a significant impact, either as primary treatment or as an adjuvant to resection. This report reviews the relevant literature pertaining to IHC with emphasis on clinical presentation, radiologic evaluation, treatment and outcome.