The first 60 days: Extrahepatic cholangiocarcinoma (perihilar and distal)
Extrahepatic cholangiocarcinoma blocks the main bile ducts outside the liver and shows itself as jaundice. Perihilar tumours need part of the liver removed with the duct and distal tumours a Whipple operation; where surgery is impossible, stenting relieves the jaundice and chemotherapy with immunotherapy follows, with HER2-directed antibodies for the one in six tumours that carry that target. Below, week by week, is what OnCo's record of Extrahepatic cholangiocarcinoma (perihilar and distal) 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.
MRI with cholangiography and CT for staging, endoscopic brushing or biopsy, and biliary drainage by stent or percutaneous route with antibiotics for cholangitis.
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 jaundice.
- RadiologistNamed in the standard of care for: Diagnosis and jaundice.
- SurgeonNamed in the standard of care for: Diagnosis and jaundice, Resectable perihilar, Resectable distal, Unresectable perihilar, selected.
- Medical oncologistNamed in the standard of care for: Resectable perihilar, Resectable distal, Unresectable perihilar, selected, Advanced, first line and 2 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Unresectable perihilar, selected.
- Transplant and cell therapy teamNamed in the standard of care for: Unresectable perihilar, selected.
- Palliative and supportive care teamNamed in the standard of care for: Diagnosis and jaundice.
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.
Bile duct resection with hemihepatectomy and caudate lobectomy after portal vein embolisation and drainage where needed, then six months of capecitabine (BILCAP).
Pancreaticoduodenectomy (Whipple) with lymphadenectomy, then adjuvant capecitabine.
Neoadjuvant chemoradiation followed by liver transplantation under the Mayo protocol in specialist centres.
Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).
Zanidatamab (HERIZON-BTC-01) or trastuzumab deruxtecan; zanidatamab first line in HERIZON-BTC-302.
FOLFOX (ABC-06); pembrolizumab for microsatellite-unstable tumours, dabrafenib-trametinib for BRAF V600E.
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 Bismuth-Corlette type and vascular involvement on MRI and CT, CA 19-9 after biliary decompression, HER2 amplification and overexpression, KRAS and TP53, Microsatellite instability and BRAF V600E), 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 Perihilarcholangiocarcinoma, Bismuth-Corlette types I to IV, Distalcholangiocarcinoma, HER2-positive extrahepatic cholangiocarcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis and jaundice
- For my situation (diagnosis and jaundice), which of the standard options do you recommend and why?Guideline options include: MRI with cholangiography and CT for staging, endoscopic brushing or biopsy, and biliary drainage by stent or percutaneous route with antibiotics for cholangitis.
Resectable perihilar
- For my situation (resectable perihilar), which of the standard options do you recommend and why?Guideline options include: Bile duct resection with hemihepatectomy and caudate lobectomy after portal vein embolisation and drainage where needed, then six months of 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.
Resectable distal
- For my situation (resectable distal), which of the standard options do you recommend and why?Guideline options include: Pancreaticoduodenectomy (Whipple) with lymphadenectomy, then adjuvant capecitabine.
- 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 perihilar, selected
- For my situation (unresectable perihilar, selected), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant chemoradiation followed by liver transplantation under the Mayo protocol in specialist centres.
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, 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 (HERIZON-BTC-01) or trastuzumab deruxtecan; zanidatamab first line in HERIZON-BTC-302.
- 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.
- How do the results of HERIZON-BTC-302 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); pembrolizumab for microsatellite-unstable tumours, dabrafenib-trametinib for BRAF V600E.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), Pembrolizumab, Dabrafenib + trametinib, 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 Zanidatamab, HERIZON-BTC-302, Trastuzumab deruxtecan, Biliary stenting and drainage?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 perihilar tumours are unresectable at presentation, and half of resections leave microscopic disease”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Biliary obstruction and cholangitis, not the cancer's growth, often end treatment”. 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.
- Extrahepatic cholangiocarcinoma (perihilar and distal): the full pageExtrahepatic cholangiocarcinoma blocks the main bile ducts outside the liver and shows itself as jaundice. Perihilar tumours need part of the liver removed with the duct and distal tumours a Whipple operation; where surgery is impossible, stenting relieves the jaundice and chemotherapy with immunotherapy follows, with HER2-directed antibodies for the one in six tumours that carry that target.
- 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.
- 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.
- CA 19-9: A sugar molecule shed into the blood by most pancreatic cancers; useful to follow treatment, not to screen.
- 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.
- Endoscopy (EGD, EUS, ERCP): Looking inside a hollow organ with a camera on a flexible tube, taking biopsies and sometimes treating on the spot.
- Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR): Microsatellite instability is the mark of a broken DNA spell-checker (loss of MLH1, MSH2, MSH6 or PMS2) that leaves thousands of mutations, so the tumour displays abnormal proteins that T cells can recognise.
Every term links to the glossary.