The first 60 days: Biliary tract cancer (cholangiocarcinoma)
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. Below, week by week, is what OnCo's record of Biliary tract cancer (cholangiocarcinoma) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
- Diagnosis and stagingNCCN category Molecular testing recommended (category 2A), NCCN Biliary Tract Cancers 2026
Contrast CT/MRI with MRCP; ERCP or EUS-guided biopsy; molecular profiling (DNA + RNA NGS) for all advanced disease; biliary drainage if jaundiced.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis and staging, Advanced, HER2-positive after chemotherapy.
- RadiologistNamed in the standard of care for: Diagnosis and staging.
- SurgeonNamed in the standard of care for: Resectable, Diagnosis and staging, Unresectable perihilar in selected patients.
- Medical oncologistNamed in the standard of care for: Advanced, Unresectable perihilar in selected patients, Advanced, first line, Advanced, FGFR2 fusion after chemotherapy and 5 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Unresectable perihilar in selected patients, Locoregional (intrahepatic, liver-confined).
- Transplant and cell therapy teamNamed in the standard of care for: Unresectable perihilar in selected patients.
- Palliative and supportive care teamNamed in the standard of care for: Diagnosis and staging.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Surgery + adjuvant capecitabine.
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.
Gem-cis + PD-(L)1; targeted therapy by genotype second line.
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.
Ivosidenib (ClarIDHy).
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).
Y-90 radioembolisation, hepatic arterial infusion pump chemotherapy, or SBRT at specialised centres; no phase 3 proof of survival benefit.
FOLFOX (ABC-06, modest benefit); liposomal irinotecan/5-FU had conflicting results (NIFTY positive, NALIRICC negative); clinical trials preferred.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example FGFR2 fusions, IDH1, HER2, NRG1, MSI), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Intrahepatic cholangiocarcinoma, Perihilarcholangiocarcinoma, Distal extrahepatic cholangiocarcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Resectable
- For my situation (resectable), which of the standard options do you recommend and why?Guideline options include: Surgery + adjuvant capecitabine.
- For my situation (resectable), which of the standard options do you recommend and why?Guideline options include: Margin-negative resection (hepatectomy, Whipple, or radical cholecystectomy) with lymphadenectomy; adjuvant capecitabine 6 months (BILCAP).
- How do the results of BILCAP apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced
- For my situation (advanced), which of the standard options do you recommend and why?Guideline options include: Gem-cis + PD-(L)1; targeted therapy by genotype second line.
- Am I a candidate for Durvalumab, Pembrolizumab, Zanidatamab or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Contrast CT/MRI with MRCP; ERCP or EUS-guided biopsy; molecular profiling (DNA + RNA NGS) for all advanced disease; biliary drainage if jaundiced.
Unresectable perihilar in selected patients
- For my situation (unresectable perihilar in selected patients), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant chemoradiation then liver transplantation (Mayo protocol) at experienced centres.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Gemcitabine-cisplatin + durvalumab (TOPAZ-1) or + pembrolizumab (KEYNOTE-966); zanidatamab added in HER2+ disease within HERIZON-BTC-302.
- Am I a candidate for Gemcitabine + cisplatin, Durvalumab, Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of TOPAZ-1 and KEYNOTE-966 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced, FGFR2 fusion after chemotherapy
- For my situation (advanced, fgfr2 fusion after chemotherapy), which of the standard options do you recommend and why?Guideline options include: Pemigatinib or futibatinib; tinengotinib in FIRST-308 after progression.
- Am I a candidate for Pemigatinib, Futibatinib, Tinengotinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of FIGHT-202 and FOENIX-CCA2 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced, IDH1 mutation after chemotherapy
- For my situation (advanced, idh1 mutation after chemotherapy), which of the standard options do you recommend and why?Guideline options include: Ivosidenib (ClarIDHy).
- Am I a candidate for Ivosidenib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ClarIDHy apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced, HER2-positive after chemotherapy
- For my situation (advanced, her2-positive after chemotherapy), which of the standard options do you recommend and why?Guideline options include: Zanidatamab (IHC 3+ or amplified); trastuzumab deruxtecan (IHC 3+, tumour-agnostic).
- Am I a candidate for Zanidatamab, Trastuzumab deruxtecan, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, other alterations
- For my situation (advanced, other alterations), which of the standard options do you recommend and why?Guideline options include: Zenocutuzumab (NRG1 fusion, approved 2026); dabrafenib-trametinib (BRAF V600E); pembrolizumab or dostarlimab (MSI-H/dMMR); larotrectinib/entrectinib (NTRK).
- Am I a candidate for Zenocutuzumab, Dabrafenib + trametinib, Pembrolizumab or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Second-line, no target
- For my situation (second-line, no target), which of the standard options do you recommend and why?Guideline options include: FOLFOX (ABC-06, modest benefit); liposomal irinotecan/5-FU had conflicting results (NIFTY positive, NALIRICC negative); clinical trials preferred.
- How do the results of NALIRICC (AIO) apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Locoregional (intrahepatic, liver-confined)
- For my situation (locoregional (intrahepatic, liver-confined)), which of the standard options do you recommend and why?Guideline options include: Y-90 radioembolisation, hepatic arterial infusion pump chemotherapy, or SBRT at specialised centres; no phase 3 proof of survival benefit.
Any stage
- Are there clinical trials I could join, for example of Zenocutuzumab, SHR-8068, TQB2102, D07001?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?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?Supportive care improves quality of life and helps patients complete treatment.
- I read that “FGFR inhibitor resistance”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Late diagnosis”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- A Clinical Study of TQB3454 Tablets in the Treatment of Advanced Biliary CarcinomaPhase 3 · active · NCT05987358A Randomized, Double-blind, Placebo-controlled, Multicenter Phase III Study to Evaluate the Efficacy and Safety of TQB3454 Tablets in the Treatment of Advanced Biliary Tract Cancer With Isocitrate Dehydrogenase 1 (IDH1) Mutation
- A Phase III Study of SHR-8068 in Combination With Adebrelimab and Platinum-Containing Chemotherapy Versus Durvalumab in Combination With Platinum-Containing Chemotherapy as First-line Treatment for Advanced Biliary Tract Cancer (BTC)Phase 3 · recruiting · NCT07229625A Phase III, Randomized, Controlled, Open-Label, Multicenter Study of SHR-8068 in Combination With Adebrelimab and Platinum-Containing Chemotherapy Versus Durvalumab in Combination With Platinum-Containing Chemotherapy as First-Line Treatment for Advanced Biliary Tract Cancer (BTC)
- A Study Comparing BL-B01D1 With Treatment of Physician's Choice in Patients With Locally Advanced or Metastatic Biliary Tract Cancer After Failure of Platinum-based Chemotherapy(PANKU-BTC01)Phase 3 · recruiting · NCT07582315A Phase III Randomized Controlled Clinical Study Comparing BL-B01D1 With Treatment of Physician's Choice in Patients With Locally Advanced or Metastatic Biliary Tract Cancer After Failure of Platinum-based Chemotherapy(PANKU-BTC01)
- A Study of BL-M07D1 Versus Physician's Choice of Chemotherapy in Patients With HER2-expressing Locally Advanced or Metastatic Biliary Tract Cancer After Platinum-containing Chemotherapy FailurePhase 3 · recruiting · NCT07606599A Phase III Randomized Controlled Clinical Study of BL-M07D1 Versus Physician's Choice of Chemotherapy in Patients With HER2-expressing Locally Advanced or Metastatic Biliary Tract Cancer After Platinum-containing Chemotherapy Failure
- A Study of Rilvegostomig or Durvalumab Plus Chemotherapy for First-Line Treatment of Biliary Tract Cancer (ARTEMIDE-Biliary02)Phase 3 · recruiting · NCT07221253Phase III, Randomized, Open-label, Global, Multicenter Study of Rilvegostomig or Durvalumab in Combination With Chemotherapy as a First-line Treatment for Patients With Advanced Biliary Tract Cancer (ARTEMIDE-Biliary02)
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Biliary tract cancer (cholangiocarcinoma): the full pageCholangiocarcinoma 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- FGFR2 fusions and rearrangements: A broken-and-rejoined FGFR2 gene that drives about one in eight intrahepatic bile duct cancers and can be switched off with pills.
- Intrahepatic, perihilar, distal and gallbladder cancer: Bile duct cancers are named by where they start: inside the liver, at the hilum where the ducts join, in the lower duct near the pancreas, or in the gallbladder.
- Stenting (biliary, oesophageal, airway): Placing a small mesh or plastic tube to hold open a duct or passage that a tumour is squeezing shut, relieving jaundice, swallowing difficulty or breathlessness.
- Whipple procedure (pancreaticoduodenectomy): The big operation for cancers of the head of the pancreas: the surgeon removes the pancreatic head, the duodenum, the gallbladder and part of the bile duct, then reconnects everything.
- Hepatectomy (liver resection): Cutting out the part of the liver containing tumour.
- CA 19-9: A sugar molecule shed into the blood by most pancreatic cancers; useful to follow treatment, not to screen.
- Obstructive jaundice and biliary obstruction: Yellowing of the skin and eyes because a tumour blocks the bile duct, most often pancreatic or bile duct cancer.
- Gene fusion: A gene fusion is two genes broken and joined together, creating a hybrid protein that can drive cancer.
- Tumour-agnostic (tissue-agnostic) approval: A tumour-agnostic approval lets a drug be used for any cancer carrying a specific molecular feature, regardless of where it started.
- Endoscopy (EGD, EUS, ERCP): Looking inside a hollow organ with a camera on a flexible tube, taking biopsies and sometimes treating on the spot.
Every term links to the glossary.