The first 60 days: Intrahepatic cholangiocarcinoma
Intrahepatic cholangiocarcinoma starts in the small bile ducts inside the liver and usually appears as a liver mass rather than causing jaundice. It is the biliary cancer with the most drug targets: FGFR2 fusions treated with pemigatinib or futibatinib, IDH1 mutations with ivosidenib, and for everyone chemotherapy with the immunotherapy durvalumab or pembrolizumab. Below, week by week, is what OnCo's record of Intrahepatic 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.
Contrast MRI and CT, biopsy, and comprehensive genomic profiling including RNA-based fusion detection for every patient.
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.
- RadiologistNamed in the standard of care for: Diagnosis.
- SurgeonNamed in the standard of care for: Resectable, Unresectable, liver-confined.
- Medical oncologistNamed in the standard of care for: Resectable, Unresectable, liver-confined, Advanced, first line, Advanced, FGFR2 fusion after chemotherapy and 2 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Unresectable, liver-confined.
- Transplant and cell therapy teamNamed in the standard of care for: Unresectable, liver-confined.
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.
Hepatectomy with lymph node dissection followed by six months of adjuvant capecitabine (BILCAP).
Radioembolisation, stereotactic radiotherapy or hepatic artery infusion in specialised centres, usually with systemic therapy; liver transplantation in trials.
Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).
Pemigatinib (FIGHT-202) or futibatinib (FOENIX-CCA2); management of hyperphosphataemia and eye toxicity.
Ivosidenib (ClarIDHy).
FOLFOX (ABC-06); trials.
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 and rearrangements, IDH1 mutation, BAP1, ARID1A and PBRM1, HER2, BRAF V600E, NRG1 and microsatellite instability, CA 19-9 for monitoring), 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 Mass-forming intrahepatic cholangiocarcinoma, Intrahepatic cholangiocarcinoma with FGFR2 fusion, Intrahepatic cholangiocarcinoma with IDH1 mutation.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: Contrast MRI and CT, biopsy, and comprehensive genomic profiling including RNA-based fusion detection for every patient.
Resectable
- For my situation (resectable), which of the standard options do you recommend and why?Guideline options include: Hepatectomy with lymph node dissection followed by six months of adjuvant capecitabine (BILCAP).
- Am I a candidate for Capecitabine, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of BILCAP apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Unresectable, liver-confined
- For my situation (unresectable, liver-confined), which of the standard options do you recommend and why?Guideline options include: Radioembolisation, stereotactic radiotherapy or hepatic artery infusion in specialised centres, usually with systemic therapy; liver transplantation in trials.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).
- 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 (FIGHT-202) or futibatinib (FOENIX-CCA2); management of hyperphosphataemia and eye toxicity.
- Am I a candidate for Pemigatinib, Futibatinib, 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.
Second line without a target
- For my situation (second line without a target), which of the standard options do you recommend and why?Guideline options include: FOLFOX (ABC-06); trials.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Tinengotinib, FIRST-308, ctDNA-guided switching among FGFR inhibitors, Liquid biopsy (ctDNA)?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 “Most patients present unresectable, and recurrence after resection is common”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “FGFR inhibitor resistance develops within a year through kinase domain mutations”. 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.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Intrahepatic cholangiocarcinoma: the full pageIntrahepatic cholangiocarcinoma starts in the small bile ducts inside the liver and usually appears as a liver mass rather than causing jaundice. It is the biliary cancer with the most drug targets: FGFR2 fusions treated with pemigatinib or futibatinib, IDH1 mutations with ivosidenib, and for everyone chemotherapy with the immunotherapy durvalumab or pembrolizumab.
- 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.
- Next-generation sequencing (NGS): Reading millions of DNA fragments in parallel, the engine behind every modern genomic test.
- Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
- Hepatectomy (liver resection): Cutting out the part of the liver containing tumour.
Every term links to the glossary.