Cholangiocarcinoma: a compact review of the literature

Abstract

Cholangiocarcinoma (CC) is a devastating cancer arising from biliary epithelia. Unfortunately, the incidence of this disease is increasing in Western countries. These tumors progress insidiously, and liver failure, biliary sepsis, malnutrition and cancer cachexia are general modes of death associated with this disease. To date, no established therapy for advanced disease has been established or validated. However, our knowledge in tumor biology is increasing dramatically and new drugs are under investigation for treatment of this notorious tumor. In clinical practice, there are better diagnostic tools in use to facilitate an earlier diagnosis of CC, at least in those patients with known risk factors. CC is resectable for cure in only a small percentage of patients. Preoperative staging for vascular and biliary extension of CC is very important in this tumor. Laparoscopy and recently endosonography seem to protect against unnecessary laparotomies in these patients. During the last 15 years, aggressive surgical approaches, including combined liver resections and vascular reconstructive surgical expertise, have improved survival in patients with CC. Surgery is contraindicated in CC cases having primary sclerosing cholangitis (PSC). Although CC was previously considered a contraindication to liver transplantation, new cautious protocols, including neo-adjuvant chemoradiation therapies and staging procedures before the transplantation, have made it possible to achieve long-term survival after liver transplantation in this disease. New ablative therapies with photodynamic therapy, intraductal high-intensity ultrasonography and chemotherapy-impregnated plastic biliary endoprosthesis are important steps in the palliative management of extra-hepatic CCs. Radiofrequency and chemo-embolization methods are also applicable for intra-hepatic CCs as palliative modes of treatment. We need more prospective randomized controlled trials to evaluate the role of the new emerging therapies for CC patients.

Figure 1

Hilar lesions are further subdivided based on location as indicated by Bismuth-Corlette classification. A: Bismuth-Corlette classification of hilar CC. TypeIinvolves the common hepatic duct, distal to the bifurcation of the biliary tree. Type II affects the bifurcation. Type IIIa affects the right hepatic duct in addition to the bifurcation. Type IIIb affects the left hepatic duct in addition to the bifurcation. Type IV involves the bifurcation and both right and left hepatic ducts or indicates multifocal CC; B: Endoscopic retrograde cholangiography reveals hilar cholangiocarcinoma (Klatskin type 2) in one of our cases.