RESUMO
Cholangiocarcinoma (CCA) is the second most common type of primary liver cancer and includes a group of intra- and extrahepatic bile tract cancers. Prognosis of patients with CCA remains poor due to high recurrence rates after curative resections and often late diagnosis in advanced stages of the disease. Curative therapy is a complete resection that requires complex surgical procedures and potentially pre-operative induction of liver hypertrophy to ensure sufficient postoperative liver function. Adjuvant therapy with capecitabine for 6 months is well established in clinical routine. The therapeutic landscape for advanced stages is constantly progressing, due to new results of clinical phase II/III trials. On the one hand, molecular analyses have paved the way to effective targeted therapies for subgroups of CCA patients with alterations in FGFR2- or IDH1-signaling. On the other hand, immune-oncological approaches in combination with chemotherapy have resulted in safe and effective treatments for unselected patient cohorts. Further studies will investigate molecular-driven as well as immune-combination treatments in earlier stages of the disease and will result in new therapy options and better prognosis for CCA patients in the near future.