{"entity":{"id":"biliary-tract-cancer","kind":"cancer","name":"Biliary tract cancer (all types)","aka":["Biliary cancer","Cancers of the bile ducts, gallbladder and ampulla"],"tldr":"Biliary tract cancers arise in the bile ducts inside or outside the liver, the gallbladder or the ampulla where the duct meets the bowel. They share short survival and the same first-line chemotherapy with immunotherapy, but differ in causes and in the targetable mutations they carry. Each has its own page.","summary":"Biliary tract cancers are adenocarcinomas of the bile duct system. Intrahepatic cholangiocarcinoma arises within the liver, perihilar and distal cholangiocarcinoma along the main ducts, gallbladder cancer in the gallbladder and ampullary cancer at the junction with the duodenum. Causes include liver fluke infection, primary sclerosing cholangitis, gallstones and, in Chile and India, a high background rate of gallbladder cancer. Surgery is the only cure and is possible in a minority; gemcitabine-cisplatin with durvalumab or pembrolizumab is the first-line treatment for advanced disease, and intrahepatic tumours often carry FGFR2 fusions or IDH1 mutations with approved targeted drugs. The subtype pages carry the detail.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Biliary_tract_cancer","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Biliary_tract_cancer"},{"label":"NCI PDQ: bile duct cancer","url":"https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq"}],"tags":["parent-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"Around 200,000 cases a year worldwide, rare in the West and common in parts of Asia and South America; most present late and five-year survival is under 20 percent overall.","subtypes":["Cholangiocarcinoma (intrahepatic, perihilar and distal bile duct)","Gallbladder cancer","Ampullary cancer (ampulla of Vater)"],"biomarkers":["FGFR2 fusions and IDH1 mutations (intrahepatic)","HER2 amplification (gallbladder, extrahepatic)","Microsatellite instability","CA 19-9"],"standardOfCare":[{"setting":"Resectable disease","approach":"Surgery followed by six months of capecitabine (BILCAP).","refs":["bilcap","capecitabine"]},{"setting":"Advanced disease","approach":"Gemcitabine-cisplatin with durvalumab (TOPAZ-1) or pembrolizumab; targeted therapy for FGFR2 and IDH1 alterations on progression.","refs":["cholangiocarcinoma","gemcitabine","cisplatin","durvalumab"]},{"setting":"Second line by biology","approach":"Pemigatinib or futibatinib for FGFR2 fusions, ivosidenib for IDH1 mutations, FOLFOX otherwise.","refs":["pemigatinib","futibatinib","ivosidenib","oxaliplatin"]}],"stateOfArt":["Immunotherapy added to chemotherapy improved survival for the first time in a decade (TOPAZ-1, KEYNOTE-966).","FGFR2 and IDH1 inhibitors made intrahepatic cholangiocarcinoma a model for molecular selection in a rare cancer.","Liver transplantation for selected perihilar tumours and liver-directed therapy for intrahepatic disease are expanding."],"history":[{"year":1965,"title":"Klatskin describes perihilar cholangiocarcinoma","refs":[]},{"year":2010,"title":"ABC-02: gemcitabine-cisplatin becomes standard","refs":["abc-02"]},{"year":2020,"title":"Pemigatinib: first FGFR2 inhibitor approved","refs":["pemigatinib"]},{"year":2022,"title":"Durvalumab added to chemotherapy (TOPAZ-1)","refs":["durvalumab"]}],"pipeline":["pemigatinib","futibatinib","ivosidenib"],"openProblems":["Most patients are diagnosed too late for surgery.","No screening even in high-incidence regions.","Resistance to FGFR2 inhibitors within a year.","Gallbladder cancer lacks any approved targeted drug."]},"route":"/cancers/biliary-tract-cancer/","neighbours":{"trial":[{"id":"nct07729033","kind":"trial","name":"A Phase II Exploratory Study of Sacituzumab Tirumotecan Combined With Envafolimab for TROP2-Positive Advanced Biliary Tract Cancers Previously Treated With First-Line 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