{"entity":{"id":"extrahepatic-cholangiocarcinoma","kind":"cancer","name":"Extrahepatic cholangiocarcinoma (perihilar and distal)","aka":["Perihilar cholangiocarcinoma","Klatskin tumour","Hilar cholangiocarcinoma","Distal cholangiocarcinoma","Common bile duct cancer","eCCA"],"tldr":"Extrahepatic cholangiocarcinoma blocks the main bile ducts outside the liver and shows itself as jaundice. Perihilar tumours need part of the liver removed with the duct and distal tumours a Whipple operation; where surgery is impossible, stenting relieves the jaundice and chemotherapy with immunotherapy follows, with HER2-directed antibodies for the one in six tumours that carry that target.","summary":"Extrahepatic cholangiocarcinoma is divided at the cystic duct into perihilar tumours, described by Klatskin in 1965 and classified by Bismuth and Corlette according to how far they extend into the right and left hepatic ducts, and distal tumours of the common bile duct. Both present with painless jaundice, pale stools, dark urine and itching, often with cholangitis, and CA 19-9 is raised but unreliable in the presence of obstruction. Primary sclerosing cholangitis, choledochal cysts and liver flukes are risk factors. The genome differs from intrahepatic disease: KRAS and TP53 mutations dominate, HER2 amplification occurs in about one in six, and FGFR2 fusions and IDH1 mutations are rare.\n\nSurgery is the only cure and is among the most demanding in abdominal oncology: perihilar tumours require resection of the bile duct with the ipsilateral hemiliver and caudate lobe, often after portal vein embolisation to grow the remnant and biliary drainage to reverse jaundice, while distal tumours are removed by pancreaticoduodenectomy. Clear margins are achieved in perhaps half of cases. Adjuvant capecitabine for six months follows BILCAP. For unresectable perihilar tumours in primary sclerosing cholangitis or under 3 cm, the Mayo protocol of chemoradiation followed by liver transplantation achieves long-term survival in selected patients and is offered in a few centres.\n\nUnresectable disease is treated with gemcitabine and cisplatin plus durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966), the biliary standards since 2022; FOLFOX is the second line after ABC-06, and liposomal irinotecan failed in NALIRICC. HER2-positive tumours respond to zanidatamab, which had a response rate of 41 percent in HERIZON-BTC-01 and gained accelerated FDA approval in 2024, and to trastuzumab deruxtecan; the phase 3 HERIZON-BTC-302 tests zanidatamab in the first line. Biliary stenting, endoscopic or percutaneous, and treatment of cholangitis are as important to survival as the anticancer drugs, since obstruction and infection are what usually end treatment.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Klatskin_tumor","links":[{"label":"TOPAZ-1 (NEJM Evidence 2022)","url":"https://evidence.nejm.org/doi/full/10.1056/EVIDoa2200015"},{"label":"HERIZON-BTC-01 (Lancet Oncology 2023)","url":"https://pubmed.ncbi.nlm.nih.gov/37276871/"},{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Klatskin_tumor"}],"tags":["subtype-page"],"related":["intrahepatic-cholangiocarcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":["abc-06"],"people":[],"bottlenecks":[],"keyPapers":["paper-bilcap-lancet-oncol-2019","paper-abc-02-gemcitabine-cisplatin-nejm-2010","paper-topaz-1-nejm-evidence-2022"],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"Cancers of the bile ducts outside the liver, from the hilum where the ducts join to the lower duct near the pancreas; perihilar tumours are the commonest cholangiocarcinoma overall, and they present with jaundice, which brings both early symptoms and the risks of biliary obstruction.","subtypes":["Perihilar (Klatskin) cholangiocarcinoma, Bismuth-Corlette types I to IV","Distal (common bile duct) cholangiocarcinoma","HER2-positive extrahepatic cholangiocarcinoma (zanidatamab)","Extrahepatic cholangiocarcinoma in primary sclerosing cholangitis (transplant protocols)","Periductal-infiltrating versus papillary growth"],"biomarkers":["Bismuth-Corlette type and vascular involvement on MRI and CT (resectability)","CA 19-9 after biliary decompression","HER2 amplification and overexpression (about one in six)","KRAS and TP53 (prognostic)","Microsatellite instability and BRAF V600E (rare, actionable)","Bilirubin and future liver remnant volume before surgery"],"standardOfCare":[{"setting":"Diagnosis and jaundice","approach":"MRI with cholangiography and CT for staging, endoscopic brushing or biopsy, and biliary drainage by stent or percutaneous route with antibiotics for cholangitis.","refs":["mri","ct","endoscopy","biliary-stenting-drainage","ca19-9"]},{"setting":"Resectable perihilar","approach":"Bile duct resection with hemihepatectomy and caudate lobectomy after portal vein embolisation and drainage where needed, then six months of capecitabine (BILCAP).","refs":["hepatectomy","lymphadenectomy","bilcap","capecitabine"]},{"setting":"Resectable distal","approach":"Pancreaticoduodenectomy (Whipple) with lymphadenectomy, then adjuvant capecitabine.","refs":["whipple","lymphadenectomy","bilcap","capecitabine"]},{"setting":"Unresectable perihilar, selected","approach":"Neoadjuvant chemoradiation followed by liver transplantation under the Mayo protocol in specialist centres.","refs":["liver-transplant-oncology"]},{"setting":"Advanced, first line","approach":"Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).","refs":["topaz-1","keynote-966","abc-02","gemcitabine-cisplatin","durvalumab","pembrolizumab","gemcis-plus-io-btc"]},{"setting":"Advanced, HER2-positive after chemotherapy","approach":"Zanidatamab (HERIZON-BTC-01) or trastuzumab deruxtecan; zanidatamab first line in HERIZON-BTC-302.","refs":["zanidatamab","trastuzumab-deruxtecan","her2","herizon-btc-302"]},{"setting":"Second line without a target","approach":"FOLFOX (ABC-06); pembrolizumab for microsatellite-unstable tumours, dabrafenib-trametinib for BRAF V600E.","refs":["folfox","pembrolizumab","msi","dabrafenib-trametinib"]}],"stateOfArt":["Surgery for perihilar tumours has become safer with portal vein embolisation and staged drainage, but remains the domain of a few high-volume centres.","Immunotherapy with gemcitabine-cisplatin is the first-line standard, and HER2 has become the actionable target of extrahepatic disease as FGFR2 and IDH1 are of intrahepatic.","Liver transplantation after chemoradiation offers cure to a small, carefully selected group with unresectable perihilar tumours."],"history":[{"year":1965,"title":"Klatskin describes adenocarcinoma at the hepatic duct bifurcation","refs":[]},{"year":1975,"title":"Bismuth and Corlette classify perihilar tumours by ductal extension","refs":[]},{"year":2010,"title":"ABC-02: gemcitabine plus cisplatin standard for advanced biliary cancer","refs":["abc-02","gemcitabine-cisplatin"]},{"year":2019,"title":"BILCAP: adjuvant capecitabine after resection","refs":["bilcap","capecitabine"]},{"year":2021,"title":"ABC-06: FOLFOX as second line","refs":["folfox"]},{"year":2022,"title":"TOPAZ-1: durvalumab added to gemcitabine-cisplatin","refs":["topaz-1","durvalumab"]},{"year":2024,"title":"Zanidatamab receives accelerated approval for HER2-positive biliary tract cancer","refs":["zanidatamab","her2"]}],"pipeline":["zanidatamab","herizon-btc-302","trastuzumab-deruxtecan","biliary-stenting-drainage","liver-transplant-oncology"],"openProblems":["Most perihilar tumours are unresectable at presentation, and half of resections leave microscopic disease.","Biliary obstruction and cholangitis, not the cancer's growth, often end treatment.","No targeted therapy exists for the KRAS-mutant majority."],"parent":"cholangiocarcinoma"},"route":"/cancers/extrahepatic-cholangiocarcinoma/","neighbours":{"cancer":[{"id":"cholangiocarcinoma","kind":"cancer","name":"Biliary tract cancer (cholangiocarcinoma)","route":"/cancers/cholangiocarcinoma/"},{"id":"cystic-duct-carcinoma","kind":"cancer","name":"Cystic duct carcinoma","route":"/cancers/cystic-duct-carcinoma/"},{"id":"intrahepatic-cholangiocarcinoma","kind":"cancer","name":"Intrahepatic cholangiocarcinoma","route":"/cancers/intrahepatic-cholangiocarcinoma/"}],"trial":[{"id":"nct07632235","kind":"trial","name":"A Multicenter, Prospective, Randomized, Open-label Phase Ib/II Study of Celecoxib Plus Pembrolizumab and Gemcitabine/Cisplatin Versus Pembrolizumab and Gemcitabine/Cisplatin in Patients With CK5/6-High Unresectable Locally Advanced or Metastatic Intrahepatic Cholangiocarcinoma","route":"/trials/nct07632235/"},{"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 Immunotherapy Combined With Chemotherapy.","route":"/trials/nct07729033/"},{"id":"nct07623642","kind":"trial","name":"A Phase II Study of GV20-0251 in Combination With Anti-PD-1 Monoclonal Antibodies in Patients With Unresectable, Locally Advanced, or Metastatic Solid Tumors.","route":"/trials/nct07623642/"},{"id":"nct07269158","kind":"trial","name":"A Phase IIb Randomized Clinical Trial of Immune Checkpoint Inhibitor-based Maintenance Therapy in Patients With Advanced Biliary Tract Cancer","route":"/trials/nct07269158/"},{"id":"nct07471165","kind":"trial","name":"A Prospective, Single-Arm, Phase II Study: PD-L1 Monoclonal Antibody + Chemoradiotherapy as Bridge Therapy to Liver Transplantation for Locally Advanced Perihilar Cholangiocarcinoma (ACHIEVE-LT)","route":"/trials/nct07471165/"},{"id":"nct07099794","kind":"trial","name":"A Randomized, Two-cohort, Prospective Phase II Clinical Study 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