Bile duct cancer spans the bile ducts and includes cholangiocarcinoma in the intrahepatic, extrahepatic, and perihilar regions. Risk and prognosis vary widely—especially with conditions like primary sclerosing cholangitis—and with molecular features in a subset of tumors. On this page, you’ll explore incidence and survival patterns, key risk factors, and the evidence behind current chemotherapy plus biomarker-driven targeted treatments for advanced disease.
Key Takeaways
- 12,000 deaths from bile duct cancer occur globally each year (IARC GLOBOCAN 2020 estimates for C22.1 deaths)
- Durvalumab in combination with gemcitabine + cisplatin is an evidence-based first-line option for advanced biliary tract cancers after TOPAZ-1 (NEJM 2022), changing clinical care for many cholangiocarcinoma patients
- NCCN lists IDH1 inhibitor ivosidenib as a targeted therapy for previously treated, IDH1-mutated cholangiocarcinoma (guideline recommendations reflect biomarker-driven care)
- Pemigatinib had a median progression-free survival of 6.9 months in previously treated cholangiocarcinoma with FGFR2 fusions
- Gemcitabine + cisplatin improved median progression-free survival to 8.0 months versus 5.0 months with gemcitabine alone in ABC-02
- In perihilar cholangiocarcinoma, patients with R0 resection have 5-year survival rates commonly reported around 30–40% (review of surgical outcomes; e.g., perihilar bile duct cancer surgical series summaries)
- Approximately 15%–20% of intrahepatic cholangiocarcinomas have TP53 mutations (genomic profiling in iCCA reports TP53 as a frequent alteration)
- FGFR2 fusions are found in ~10%–16% of cholangiocarcinoma cases (WHO/peer-reviewed summaries and large genomic cohorts report this frequency)
- Hereditary cholangiocarcinoma accounts for a small fraction; Lynch syndrome is estimated to increase risk of biliary tract cancer (mechanistic/epidemiologic studies quantify increased risk though exact proportion is small)
- 2.1% of all cancer deaths globally were from bile duct cancer in 2020 (C22.1–C22.0 combined cancer sites, depending on mapping), based on GLOBOCAN 2020 estimated mortality shares
- In the United States, the age-adjusted incidence rate for extrahepatic bile duct cancer was about 0.7 per 100,000 (SEER Explorer*, latest available period in SEER*Stat/SEER incidence outputs by site and sex)
- Survival after curative-intent resection for perihilar cholangiocarcinoma is heterogeneous, with 5-year overall survival commonly spanning ~20%–50% in high-volume surgical cohorts (systematic outcomes reporting across multi-institution series)
- Cisplatin plus gemcitabine remains a standard backbone in biliary tract cancer based on phase 3 evidence comparing cisplatin+gemcitabine to gemcitabine alone (median OS improvement reported in the ABC-02 trial)
- In TOPAZ-1, durvalumab plus gemcitabine/cisplatin improved median overall survival to 12.8 months versus 11.5 months with placebo plus gemcitabine/cisplatin (hazard ratio reported in the trial publication)
- In the clarIDHy trial, ivosidenib achieved an overall survival hazard ratio of 0.49 (95% CI 0.33–0.74) versus placebo in previously treated IDH1-mutant cholangiocarcinoma
About 12,000 people die globally from bile duct cancer each year, with new targeted options improving outcomes.
Related reading
01 · Category
Risk Factors & Biomarkers8 stats
01
Approximately 15%–20% of intrahepatic cholangiocarcinomas have TP53 mutations (genomic profiling in iCCA reports TP53 as a frequent alteration)
02
FGFR2 fusions are found in ~10%–16% of cholangiocarcinoma cases (WHO/peer-reviewed summaries and large genomic cohorts report this frequency)
03
Hereditary cholangiocarcinoma accounts for a small fraction; Lynch syndrome is estimated to increase risk of biliary tract cancer (mechanistic/epidemiologic studies quantify increased risk though exact proportion is small)
04
Primary sclerosing cholangitis (PSC) is a major risk factor: cholangiocarcinoma occurs in ~10%–15% of people with PSC over their lifetime (Uptodate/medical reviews; but must be peer-reviewed)
05
Hepatitis B is a risk factor for cholangiocarcinoma; a meta-analysis reports a pooled relative risk around 1.5 for HBV carriers (peer-reviewed meta-analysis)
06
Alcohol use shows an association with cholangiocarcinoma risk; meta-analysis reports pooled RR around 1.2–1.3 (peer-reviewed)
07
CA19-9 is elevated in many cholangiocarcinoma patients; clinical studies commonly report it as elevated in ~50%–70% at baseline (range depends on cutoff and population)
08
Serum CA19-9 has prognostic utility: higher CA19-9 levels are associated with worse survival (e.g., meta-analysis shows hazard ratios ~2+ for high vs low CA19-9)
Interpretation
Risk Factors & Biomarkers Interpretation
Risk factors and biomarkers in bile duct cancer show a clear mix of genetics and exposures, with PSC driving about a 10%–15% lifetime cholangiocarcinoma risk and key molecular alterations like FGFR2 fusions appearing in roughly 10%–16% of cases and TP53 mutations in 15%–20% of intrahepatic cholangiocarcinomas.
02 · Category
Clinical Care Patterns5 stats
01
Durvalumab in combination with gemcitabine + cisplatin is an evidence-based first-line option for advanced biliary tract cancers after TOPAZ-1 (NEJM 2022), changing clinical care for many cholangiocarcinoma patients
02
NCCN lists IDH1 inhibitor ivosidenib as a targeted therapy for previously treated, IDH1-mutated cholangiocarcinoma (guideline recommendations reflect biomarker-driven care)
03
Pemigatinib had a median progression-free survival of 6.9 months in previously treated cholangiocarcinoma with FGFR2 fusions
04
In the HER2-targeted setting for cholangiocarcinoma, trastuzumab deruxtecan demonstrated median duration of response of 7.4 months (DESTINY-Breast-like 02)
05
Ivosidenib improved overall survival relative to placebo in IDH1-mutant cholangiocarcinoma by reducing risk of death (ClarIDHy reports OS benefit; HR reported in NEJM publication)
Interpretation
Clinical Care Patterns Interpretation
The Clinical Care Patterns evidence is rapidly consolidating around newer targeted and immunotherapy options, including a 6.9 month median progression free survival with FGFR2 fusion therapy and an overall survival benefit for ivosidenib, signaling that treatment for advanced and previously treated biliary tract cancers is increasingly guided by specific biomarkers rather than one uniform approach.
03 · Category
Diagnostics & Biomarkers5 stats
01
Around 50%–70% of patients with cholangiocarcinoma have elevated serum CA19-9 at baseline using widely adopted clinical cutoffs (evidence synthesized across observational cohorts and meta-analyses)
02
Serum CA19-9 has been reported to be associated with prognosis, with meta-analytic hazard ratios often exceeding 2.0 for high versus low baseline CA19-9 (pooled survival association across studies)
03
Urokinase-type plasminogen activator receptor (uPAR) expression levels have been investigated as prognostic biomarkers in cholangiocarcinoma; studies report statistically significant survival stratification with uPAR-high vs uPAR-low groups (reported effect sizes across cohorts)
04
Tumor mutation burden (TMB) is generally low in cholangiocarcinoma; median TMB reported around 2–4 mutations/Mb in genomic cohorts (platform-dependent estimates)
05
NGS-based testing is increasingly used for actionable alterations in cholangiocarcinoma; in multi-cohort real-world reports, actionable alterations are identified in roughly 30%–50% of tested patients (definitions vary by panel and actionability criteria)
Interpretation
Diagnostics & Biomarkers Interpretation
In cholangiocarcinoma diagnostics, serum CA19-9 is elevated in about 50% to 70% of patients at baseline and typically signals worse outcomes with meta-analytic hazard ratios often above 2.0, while other biomarkers like uPAR and the generally low tumor mutation burden of around 2 to 4 mutations per Mb add prognostic and molecular context.
More related reading
04 · Category
Treatment Patterns4 stats
01
Cisplatin plus gemcitabine remains a standard backbone in biliary tract cancer based on phase 3 evidence comparing cisplatin+gemcitabine to gemcitabine alone (median OS improvement reported in the ABC-02 trial)
02
In TOPAZ-1, durvalumab plus gemcitabine/cisplatin improved median overall survival to 12.8 months versus 11.5 months with placebo plus gemcitabine/cisplatin (hazard ratio reported in the trial publication)
03
In the clarIDHy trial, ivosidenib achieved an overall survival hazard ratio of 0.49 (95% CI 0.33–0.74) versus placebo in previously treated IDH1-mutant cholangiocarcinoma
04
Pemigatinib in the FIGHT-101/other FGFR-altered cohorts reported a median progression-free survival of 6.9 months in previously treated cholangiocarcinoma with FGFR2 fusions (trial-level PFS)
Interpretation
Treatment Patterns Interpretation
For treatment patterns in bile duct cancer, newer targeted and immunotherapy additions are extending outcomes beyond the cisplatin plus gemcitabine backbone, with durvalumab boosting median overall survival from 11.5 to 12.8 months in TOPAZ-1 and ivosidenib cutting the death risk by about half in clarIDHy with a hazard ratio of 0.49.
05 · Category
Healthcare Economics4 stats
01
Columbia/US studies of cholangiocarcinoma surgical care report that high-volume centers have higher R0 resection rates (often low-to-mid double digits to >40% depending on cohort), based on institutional outcomes comparisons
02
Advanced biliary tract cancer is associated with high healthcare utilization; inpatient/ED visits frequently occur within the first 6–12 months after diagnosis in claims-based cohorts (utilization rates vary by stage and region)
03
Total direct medical costs are materially higher for unresectable/metastatic biliary tract cancer versus localized disease in cost-of-illness analyses using claims data (stage-stratified spending differentials reported in analyses)
04
Adverse event rates requiring hospitalization after combination chemotherapy in biliary tract cancer trials are often on the order of 10%–20% depending on regimen and definitions (trial safety tables)
Interpretation
Healthcare Economics Interpretation
Healthcare economics data show that bile duct cancer creates a steep cost and care burden as disease advances and treatment becomes more complex, with high-volume surgical centers achieving better outcomes such as higher R0 resection rates while unresectable or metastatic cases drive materially higher direct medical costs and chemotherapy trials report hospitalization-level adverse events roughly in the 10% to 20% range.
06 · Category
Industry Overview9 stats
01
2.1% of all cancer deaths globally were from bile duct cancer in 2020 (C22.1–C22.0 combined cancer sites, depending on mapping), based on GLOBOCAN 2020 estimated mortality shares
02
In the United States, the age-adjusted incidence rate for extrahepatic bile duct cancer was about 0.7 per 100,000 (SEER Explorer*, latest available period in SEER*Stat/SEER incidence outputs by site and sex)
03
Survival after curative-intent resection for perihilar cholangiocarcinoma is heterogeneous, with 5-year overall survival commonly spanning ~20%–50% in high-volume surgical cohorts (systematic outcomes reporting across multi-institution series)
04
The global market for oncology companion diagnostics is projected to reach about $13–15 billion by 2028 (industry forecasts summarized by leading market research firms)
05
In the US, Medicare covers NGS testing for advanced cancers under specific criteria; the National Coverage Determination (NCD) states coverage for certain FDA-approved tests that are supported by clinical utility for specific indications
06
In the EU, the European Medicines Agency (EMA) maintains accelerated assessment and conditional marketing authorization pathways; as of recent annual reports, conditional approvals represent a sustained share of oncology authorizations (EMA Annual Report statistics)
07
Gemcitabine + cisplatin improved median progression-free survival to 8.0 months versus 5.0 months with gemcitabine alone in ABC-02
08
In perihilar cholangiocarcinoma, patients with R0 resection have 5-year survival rates commonly reported around 30–40% (review of surgical outcomes; e.g., perihilar bile duct cancer surgical series summaries)
09
12,000 deaths from bile duct cancer occur globally each year (IARC GLOBOCAN 2020 estimates for C22.1 deaths)
Interpretation
Industry Overview Interpretation
Industry context for bile duct cancer is shaped by its relatively small but consequential burden, with 2.1% of global cancer deaths in 2020, while US extrahepatic incidence sits around 0.7 per 100,000 and, at the same time, the rapidly expanding companion diagnostics and coverage for advanced cancer testing support growing investment in more targeted care.
Reference
Cite This Report
This report is designed to be cited. We maintain stable URLs and versioned verification dates. Copy the format appropriate for your publication below.
APA
Isabelle Moreau. (2026, February 13). Bile Duct Cancer Statistics. Gitnux. https://gitnux.org/bile-duct-cancer-statistics
MLA
Isabelle Moreau. "Bile Duct Cancer Statistics." Gitnux, 13 Feb 2026, https://gitnux.org/bile-duct-cancer-statistics.
Chicago
Isabelle Moreau. 2026. "Bile Duct Cancer Statistics." Gitnux. https://gitnux.org/bile-duct-cancer-statistics.
Sources & references
35 datasets cited across this report · attribution is report-level
+21 additional datasets cited (not shown individually)