Cholangiocarcinoma is a malignant disease of the biliary ductal system which consists of intrahepatic (periphery) 5–10% and extrahepatic, which is further divided into proximal (perihilar) 60–70% and distal 20–30%. The etiology of this grave disease is unknown although many causative factors, including infectious, congenital, and genetic factors, causing chronic inflammation, which results in dysplastic changes of the biliary epithelium and eventual malignancy, have been implicated. The prognosis is poor except when discovered early. The treatment of intrahepatic (CCA) is partial hepatectomy, while radical bile duct resection with or without hepatectomy or pancreaticoduodenectomy is considered for extrahepatic cancer. Liver transplantation is considered in advanced diseases, without extrahepatic lymph node involvement. Palliation including endoscopic drainage or surgical bypass which is an option for unresectable diseases. Adjuvant therapy in the form of chemotherapy, immunotherapy, and photodynamic therapy is a consideration in patients with advanced disease. Many advances have been made in the treatment of cholangiocarcinoma, and hopefully long-term survival may be improved.
Part of the book: Surgical Challenges in the Management of Liver Disease