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Previous treatment nihilism of patients with advanced disease has been replaced by active clinical research with the advent of randomized clinical trials (<jats:styled-content style=\"fixed-case\">RCT<\/jats:styled-content>s) and a much greater effort at understanding molecular mechanisms underpinning <jats:styled-content style=\"fixed-case\">CCA<\/jats:styled-content>. Three <jats:styled-content style=\"fixed-case\">RCT<\/jats:styled-content>s have recently been reported evaluating adjuvant chemotherapy following curative resection; only one of these has the potential to change practice. The <jats:styled-content style=\"fixed-case\">BILCAP<\/jats:styled-content> study failed to meet its primary endpoint by intention\u2010to\u2010treat analysis; however, a survival benefit was seen in a preplanned sensitivity analysis (predominantly adjusting for lymph nodes status). This, along with the numerical difference in median overall survival has led to the uptake of adjuvant capecitabine by many clinicians. In patients with advanced disease, the only level 1 data available supports the use of cisplatin and gemcitabine for the first\u2010line treatment of patients with advanced disease; there is no established second\u2010line chemotherapy. Previous forays into targeted therapy have proven unfruitful (namely targeting the epithelial growth factor receptor and vascular endothelial growth factor pathways). An increasing number of genomic subtypes are being defined; for some of these on\u2010target therapeutic options are under active investigation. The most developed are studies targeting <jats:styled-content style=\"fixed-case\">IDH<\/jats:styled-content>\u20101 (isocitrate dehydrogenase) mutations and <jats:styled-content style=\"fixed-case\">FGFR<\/jats:styled-content>\u20102 (fibroblast growth factor receptor) fusions, with promising early results. Several other pathways are under evaluation, along with early studies targeting the immune environment; these are too premature to change practice to date. These emerging treatments are discussed.<\/jats:p>","DOI":"10.1111\/liv.14100","type":"journal-article","created":{"date-parts":[[2019,3,20]],"date-time":"2019-03-20T15:59:05Z","timestamp":1553097545000},"page":"123-142","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":84,"title":["Medical treatment for cholangiocarcinoma"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3959-4120","authenticated-orcid":false,"given":"Jorge","family":"Adeva","sequence":"first","affiliation":[{"name":"Department of Medical Oncology Hospital Universitario 12 de Octubre Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Sangro","sequence":"additional","affiliation":[{"name":"Liver Unit and HPB Oncology Area Clinica Universidad de Navarra\u2010IDISNA and CIBEREHD Pamplona 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