Cholangiocarcinomas are the second most frequent primary hepatic malignancy, and make up from 5% to 30% of malignant hepatic tumours. Hilar cholangiocarcinoma (HCC) is the most common type, and accounts for approximately 60% to 67% of all cholangiocarcinoma cases. There is not a staging system that permits us to compare all series and extract some conclusions to increase the long-survival rate in this dismal disease. Neither the extension of resection, according to the sort of HCC, is a closed topic. Some authors defend limited resection (mesohepatectomy with S1, S1 plus S4b-S5, local excision for papillary tumours, etc.) while others insist in the compulsoriness of an extended hepatic resection with portal vein bifurcation removed to reach cure. As there is not an ideal adjuvant therapy, R1 resection can be justified to prolong the survival rate. Morbidity and mortality rates changed along the last decade, but variability is the rule, with morbidity and mortality rates ranging from 14% to 76% and from 0% to 19%, respectively.
Conclusion: Surgical resection continues to be the main treatment of HCC. Negative resection margins achieved with major hepatic resections are associated with improved outcome. Preresectional management with biliary drainage, portal vein embolization and staging laparoscopy should be considered in selected patients. Additional evidence is needed to fully define the role of orthotopic liver transplant. Portal and lymph node involvement worsen the prognosis and long-term survival, and surgery is the only option that can lengthen it. Improvements in adjuvant therapy are essential for improving long-term outcome. Furthermore, the lack of effective chemotherapy drugs and radiotherapy approaches leads us to can consider R1 resection as an option, because operated patients have a longer survival rate than those who not undergo surgery.
Keywords: Cholangiocarcinoma; Klatskin tumor; Outcome; Pronostic factors; Survival rate.