Biliary tract cancer (all types)
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 a poor outlook and the same first-line chemotherapy with immunotherapy, but differ in causes and in the targetable mutations they carry. Each has its own page.
Overview
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.
State of the art
- 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.
Anatomy and lymph node drainage
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
- Pancreatic head (most PDAC)
- Body and tail
- AmpullaAmpullary cancer (ampulla of Vater)
- Islets (pancreatic NET)
- Intrahepatic ductsCholangiocarcinoma (intrahepatic, perihilar and distal bile duct)
- Perihilar (Klatskin)Cholangiocarcinoma (intrahepatic, perihilar and distal bile duct)
- Distal bile ductCholangiocarcinoma (intrahepatic, perihilar and distal bile duct)
- GallbladderGallbladder cancer
- peripancreatic
- hepatic hilar
- coeliac and superior mesenteric
Same organ: Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Neuroendocrine tumours, Gallbladder cancer, Ampullary cancer (ampulla of Vater)
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- 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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Gemcitabine-cisplatin with durvalumab (TOPAZ-1) or pembrolizumab; targeted therapy for FGFR2 and IDH1 alterations on progression.
Subtypes & biomarkers
top- Cholangiocarcinoma (intrahepatic, perihilar and distal bile duct)
- Gallbladder cancer
- Ampullary cancer (ampulla of Vater)
- FGFR2 fusions and IDH1 mutations (intrahepatic)
- HER2 amplification (gallbladder, extrahepatic)
- Microsatellite instability
- CA 19-9
How often this target appears
- 1965Klatskin describes perihilar cholangiocarcinoma
- 2010ABC-02: gemcitabine-cisplatin becomes standard
- 2020Pemigatinib: first FGFR2 inhibitor approved
- 2022Durvalumab added to chemotherapy (TOPAZ-1)
What is in development for Biliary tract cancer (all types), drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
Most patients are diagnosed too late for surgery.
No screening even in high-incidence regions.
and how the field plans to fix it →What is being done about thisFinding cancer earlierAvailable now- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
In trialsIdeas and roadmaps- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Resistance to FGFR2 inhibitors within a year.
and how the field plans to fix it →What is being done about thisResistance to treatmentAvailable now- AmivantamabApproved
- CamizestrantApproved
- Comprehensive genomic profilingStandard of care
- Liquid biopsy (ctDNA)Standard of care
- LorlatinibApproved
- MRD / molecular residual disease testingEstablished
In trials- ADC payload neutralisersPhase 1
- BGB-16673Phase 3
- Bispecific ADCPhase 3
- BRUIN CLL-321Positive
- CaDAnCe-304Recruiting
- CIRCULATE-Japan (GALAXY / VEGA / ALTAIR)Active
Ideas and roadmaps- A clone report from blood at every treatment cycle
- A fast route to the matched drug when it is licensed for another cancer
- A multi-cancer platform trial of adaptive (dose-holiday) therapy
- A national rapid research autopsy network for end-stage cancer
- A standard evolvability score for every tumour
- A standing platform trial that assigns treatment by how the tumour escaped
Background: ADC sequencing, Antigen escape (antigen loss, lineage switch), BCG-unresponsive, Castration-resistant prostate cancer (CRPC), Circulating tumour DNA (ctDNA). Also on OnCo: Resistance atlas · Lines of therapy.
Gallbladder cancer lacks any approved targeted drug.
Trials
topTrials recruiting now
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Landmark trials
Expert centres
topExpert centres
- via Biliary tract cancer (cholangiocarcinoma)
- via Biliary tract cancer (cholangiocarcinoma)
- via Biliary tract cancer (cholangiocarcinoma)
- via Biliary tract cancer (cholangiocarcinoma)
- Cancer Research UKLondon, GBvia BILCAP, Biliary tract cancer (cholangiocarcinoma), ABC-02
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia Biliary tract cancer (cholangiocarcinoma), Durvalumab
- All India Institute of Medical Sciences, New DelhiNew Delhi, INvia Biliary tract cancer (cholangiocarcinoma)
- Fundación Arturo López PérezSantiago, CLvia Biliary tract cancer (cholangiocarcinoma)
- German Breast Group (GBG)Neu-Isenburg, DEvia Durvalumab
- Homi Bhabha Cancer Hospital and Mahamana Pandit Madan Mohan Malaviya Cancer Centre, VaranasiVaranasi, INvia Biliary tract cancer (cholangiocarcinoma)
- Hospital Universitario 12 de OctubreMadrid, ESvia Durvalumab
- Indiana University Melvin and Bren Simon Comprehensive Cancer CenterIndianapolis, IN, USNCI comprehensivevia Cisplatin
- via Gemcitabine
- National Cancer Centre SingaporeSingapore, SGvia Biliary tract cancer (cholangiocarcinoma)
- via Biliary tract cancer (cholangiocarcinoma)
- Ramathibodi Hospital, Mahidol UniversityBangkok, THvia Biliary tract cancer (cholangiocarcinoma)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia Biliary tract cancer (cholangiocarcinoma)
- Zhongshan Hospital, Fudan UniversityShanghai, CNvia Biliary tract cancer (cholangiocarcinoma)
Questions to ask
topQuestions to ask your oncologist about Biliary tract cancer
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example FGFR2 fusions and IDH1 mutations, HER2 amplification, Microsatellite instability, CA 19-9), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Cholangiocarcinoma, Gallbladder cancer, Ampullary cancer.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Resectable disease
- For my situation (resectable disease), which of the standard options do you recommend and why?Why: Guideline options include: Surgery followed by six months of capecitabine (BILCAP).
- Am I a candidate for Capecitabine, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of BILCAP apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Advanced disease
- For my situation (advanced disease), which of the standard options do you recommend and why?Why: Guideline options include: Gemcitabine-cisplatin with durvalumab (TOPAZ-1) or pembrolizumab; targeted therapy for FGFR2 and IDH1 alterations on progression.
- Am I a candidate for Gemcitabine, Cisplatin, Durvalumab, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Most patients are diagnosed too late for surgery”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “No screening even in high-incidence regions”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
5targets
2drugs
5companies
4trials
2Latest papers
topQuery for this cancer: (TITLE:"Biliary tract cancer" OR ABSTRACT:"Biliary tract cancer" OR TITLE:"all types" OR ABSTRACT:"all types" OR TITLE:"Biliary cancer" OR ABSTRACT:"Biliary cancer" OR TITLE:"Cancers of the bile ducts, gallbladder and ampulla" OR ABSTRACT:"Cancers of the bile ducts, gallbladder and ampulla") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Biliary tract cancer (all types), not a curated reading list.
Similar pages
not linked directly; found by shared links- PersonJuan W. Valle
Shares ABC-02, Durvalumab, Biliary tract cancer (cholangiocarcinoma).
- TrialAK112 Combined With Chemotherapy Versus Durvalumab Combined With Chemotherapy in Advanced Biliary Tract Cancer
Shares Durvalumab, Gemcitabine, Biliary tract cancer (cholangiocarcinoma), Cisplatin.
- TrialPhase 3 Study of T-DXd and Rilvegostomig Versus SoC in Advanced HER2-expressing Biliary Tract Cancer
Shares Durvalumab, Gemcitabine, Biliary tract cancer (cholangiocarcinoma), Cisplatin.
- TrialDurvalumab With Chemotherapy as First Line Treatment in Patients With Advanced Biliary Tract Cancers (aBTCs)
Shares Durvalumab, Gemcitabine, Biliary tract cancer (cholangiocarcinoma), Cisplatin.
- TrialCosiporfin Sodium for Injection Photodynamic Therapy
Shares Gemcitabine, Biliary tract cancer (cholangiocarcinoma), Cisplatin.
- TrialFutibatinib (TAS-120) in Patients With Advanced Biliary Tract Cancer
Shares Durvalumab, Gemcitabine, Biliary tract cancer (cholangiocarcinoma), Cisplatin.
- TrialStudy of Durvalumab Versus Placebo in Combination With Definitive Chemoradiation Therapy in Patient With ESCC
Shares Capecitabine, Durvalumab, Cisplatin.
- TermCA 19-9
Shares Ampullary cancer (ampulla of Vater), Gallbladder cancer, Biliary tract cancer (cholangiocarcinoma).