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Cholangiocarcinoma is cancer of the bile ducts or gallbladder. It is rare and often found late, but it turned out to carry more targetable mutations than almost any other gastrointestinal cancer, and immunotherapy now adds to chemotherapy from the first treatment.
Biliary tract cancers comprise intrahepatic cholangiocarcinoma (iCCA, rising in incidence), perihilar and distal extrahepatic cholangiocarcinoma, and gallbladder cancer. They share late presentation (jaundice, weight loss) and dependence on surgical resection as the only cure, achievable in a minority, which keeps five-year survival under 20%. Risk factors differ by region: liver flukes and hepatolithiasis in East Asia, primary sclerosing cholangitis in the West, gallstones and chronic inflammation for gallbladder cancer. Biliary drainage is usually a prerequisite for any treatment.
The systemic landscape was gemcitabine-cisplatin alone from ABC-02 (2010) until TOPAZ-1 (durvalumab, 2022) and KEYNOTE-966 (pembrolizumab, 2023) added PD-(L)1 blockade with a modest median benefit but a doubling of two-year survival. Adjuvant capecitabine (BILCAP) is standard after resection. What sets biliary cancer apart is its genomic actionability: roughly 40% of intrahepatic tumours carry FGFR2 fusions (pemigatinib, futibatinib), IDH1 mutations (ivosidenib), HER2 amplification or overexpression (zanidatamab, trastuzumab deruxtecan), NRG1 fusions (zenocutuzumab, approved 2026), BRAF V600E, or MSI-high status, so molecular profiling at diagnosis is guideline-mandated.
The frontier is moving targeted agents into first line (HERIZON-BTC-302 for zanidatamab), overcoming FGFR-inhibitor resistance (tinengotinib in FIRST-308, lirafugratinib), ctDNA-guided sequencing, and finding a biomarker for the immunotherapy long-tail. Liver transplantation for unresectable perihilar tumours (Mayo protocol) and for selected intrahepatic disease is expanding. Open problems include second-line therapy after chemo-immunotherapy, gallbladder cancer's neglect in trials, and early detection in high-risk groups such as primary sclerosing cholangitis.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
About 210,000 cases per year worldwide; incidence of intrahepatic cholangiocarcinoma has roughly doubled in Western countries over 30 years; gallbladder cancer is endemic in Chile, northern India, and among Indigenous Americans.
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.
Same organ: Glucagonoma, VIPoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma, Adenosquamous carcinoma of the pancreas, Colloid (mucinous non-cystic) carcinoma of the pancreas, Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, Invasive carcinoma arising in an intraductal papillary mucinous neoplasm (IPMN-associated carcinoma), Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma), Solid pseudopapillary neoplasm of the pancreas
Most people reading this do not have advanced disease. The map describes what can happen over the whole course of the illness, across autopsy and registry series; today's staging scans find spread earlier, and each site has treatments, from focused radiotherapy for a few spots to drugs that reach the brain.
What helpsGemcitabine-cisplatin with durvalumab or pembrolizumab, then drugs matched to the FGFR2, IDH1, HER2 or NRG1 changes found in about 40% of intrahepatic tumours.
Nothing recorded yet.
Also on OnCo: Atlas of advanced disease · How cancer spreads: the metastasis stages.
Background: CA 19-9. Also on OnCo: Symptoms and red flags · Early detection roadmap.
Gem-cis + PD-(L)1; targeted therapy by genotype second line.
Contrast CT/MRI with MRCP; ERCP or EUS-guided biopsy; molecular profiling (DNA + RNA NGS) for all advanced disease; biliary drainage if jaundiced.
Margin-negative resection (hepatectomy, Whipple, or radical cholecystectomy) with lymphadenectomy; adjuvant capecitabine 6 months (BILCAP).
Neoadjuvant chemoradiation then liver transplantation (Mayo protocol) at experienced centres.
Gemcitabine-cisplatin + durvalumab (TOPAZ-1) or + pembrolizumab (KEYNOTE-966); zanidatamab added in HER2+ disease within HERIZON-BTC-302.
Pemigatinib or futibatinib; tinengotinib in FIRST-308 after progression.
Zanidatamab (IHC 3+ or amplified); trastuzumab deruxtecan (IHC 3+, tumour-agnostic).
Zenocutuzumab (NRG1 fusion, approved 2026); dabrafenib-trametinib (BRAF V600E); pembrolizumab or dostarlimab (MSI-H/dMMR); larotrectinib/entrectinib (NTRK).
FOLFOX (ABC-06, modest benefit); liposomal irinotecan/5-FU had conflicting results (NIFTY positive, NALIRICC negative); clinical trials preferred.
Y-90 radioembolisation, hepatic arterial infusion pump chemotherapy, or SBRT at specialised centres; no phase 3 proof of survival benefit.
Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year.
The targets of this cancer's medicines and the ones linked to it directly.
| Target / alteration | Prevalence | Measure | Source |
|---|---|---|---|
| HER2 Higher in gallbladder/extrahepatic | 10-20% | IHC 3+ or amplification | Wikipedia |
| IDH1 / IDH2 | 10-20% | IDH1 mutation (intrahepatic) | cBioPortal (TCGA) |
| FGFR2 | 10-15% | Fusion (intrahepatic) | cBioPortal (TCGA) |
How common each drug target or alteration is in this cancer. Population-level and approximate; see the target page for detail. Full matrix.
4 cell lines, 1 mouse models and 6 repositories are listed for this cancer. See them →
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fever, cough or breathlessness, rapid weight gain or swelling, bone pain, low blood pressure or reduced urine; the labels say to start steroids and monitor at the first suspicion, and the syndrome has been fatal.
Any new or worsening cough, breathlessness or fever. The label says to interrupt treatment for any suspected ILD and to permanently discontinue for grade 2 or higher.
See all on the product pages:Dabrafenib + trametinibDostarlimabDurvalumabFutibatinibGemcitabine + cisplatinIvosidenibPembrolizumabPemigatinibTinengotinibTrastuzumab deruxtecan·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Biliary tract cancer, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.