# Biliary tract cancer (all types)

Source: https://onco.cc/cancers/biliary-tract-cancer/  
OnCo record `biliary-tract-cancer` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## 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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Biliary cancer; Cancers of the bile ducts, gallbladder and ampulla
- Tags: parent-page
- 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

## Standard of care

- Resectable disease: Surgery followed by six months of capecitabine (BILCAP). ([BILCAP](https://onco.cc/trials/bilcap/), [Capecitabine](https://onco.cc/drugs/capecitabine/))
- Advanced disease: Gemcitabine-cisplatin with durvalumab (TOPAZ-1) or pembrolizumab; targeted therapy for FGFR2 and IDH1 alterations on progression. ([Biliary tract cancer (cholangiocarcinoma)](https://onco.cc/cancers/cholangiocarcinoma/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Durvalumab](https://onco.cc/drugs/durvalumab/))

## 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Biliary_tract_cancer
- Wikipedia: https://en.wikipedia.org/wiki/Biliary_tract_cancer
- NCI PDQ: bile duct cancer: https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq

## Connected records

- trials: [ABC-02](https://onco.cc/trials/abc-02/), [BILCAP](https://onco.cc/trials/bilcap/)
- drugs: [Capecitabine](https://onco.cc/drugs/capecitabine/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Durvalumab](https://onco.cc/drugs/durvalumab/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Pemigatinib](https://onco.cc/drugs/pemigatinib/)
- cancers: [Ampullary cancer (ampulla of Vater)](https://onco.cc/cancers/ampullary/), [Biliary tract cancer (cholangiocarcinoma)](https://onco.cc/cancers/cholangiocarcinoma/), [Gallbladder cancer](https://onco.cc/cancers/gallbladder/)

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JSON: https://onco.cc/api/v1/entities/biliary-tract-cancer.json