Abstract
Cholangiocarcinomas are a rare but highly fatal disease. The only curative treatment is radical surgical resection of the tumor and the regional lymph nodes. More than half of patients have irresectable disease, which implicates a median survival of < 1 year. The mainstay of palliative treatment is endoscopic or percutaneous drainage of the biliary system. In patients with good performance status, palliative chemotherapy seems to provide some survival benefit together with an improved quality of life. No standard chemotherapy has been defined but gemcitabine monotherapy or the combination of gemcitabine with platin derivates or capecitabine seems to be more effective than other protocols. Additionally, photodynamic therapy has shown promising results and radiation might be helpful for localized disease. In a very selected population liver transplantation can also be an option.