Purpose of review: Cholangiocarcinoma is an enigmatic malignancy of the biliary tract that has recently been shown to be increasing in incidence globally. There has been recent progress in identifying potential risk factors for the tumor, and in the use of emerging technologies for diagnosis and palliative treatment.
Recent findings: Hepatitis B and C, cirrhosis and alcohol have been described as risk factors for cholangiocarcinoma. A morphology-based classification for cholangiocarcinoma is proposed that will facilitate future descriptive epidemiology, diagnosis and comparative therapeutic studies. Diagnosis may be improved by new approaches to enhance the diagnostic yield and utility of biliary cytology. The role of new imaging approaches such as positron emission tomography scanning, endoscopic ultrasound or optical coherence tomography for diagnosis are being examined and defined. Long-term results for transplantation protocols for curative intent in non-resectable localized disease have been described. Photodynamic therapy looks extremely promising for adjunct therapy of intrahepatic mass lesions.
Summary: Recent advances in the epidemiology, classification, diagnosis and therapy of cholangiocarcinoma are expected to enhance the evaluation and management of patients with this devastating malignancy.