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Health condition · Clinically reviewed

Hilar cholangiocarcinoma, the Klatskin tumour - and how it is treated today.

A cancer at the confluence of the bile ducts. Curable in selected patients with surgery or transplant, and increasingly treatable with modern systemic and targeted therapy.

Jump to treatment
A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a UK hepatobiliary clinician before publication.

  • 02

    Sourced from guidance

    Checked against BSG, NICE and ILCA guidance, with the sources listed at the end.

  • 03

    Current for 2026

    Reflects modern UK practice including durvalumab, pemigatinib, ivosidenib and Y-90 radioembolisation.

Key facts

Hilar cholangiocarcinoma at a glance.

The essentials, in plain English - what it is, who gets it, and how it is treated in the UK today.

  • What it is

    A cancer of the bile ducts at the confluence of the right and left hepatic ducts, historically called a Klatskin tumour.

  • How common

    The most common subtype of cholangiocarcinoma, accounting for roughly half to two-thirds of all bile-duct cancers.

  • Classification

    The Bismuth-Corlette system (types I to IV) describes how far the tumour extends along the right and left ducts.

  • Typical presentation

    Painless jaundice with itching, weight loss and right upper quadrant discomfort - often the first clue to the diagnosis.

  • Curative option

    Major liver resection with bile-duct reconstruction, or - for selected unresectable cases - neoadjuvant chemoradiotherapy and liver transplant.

  • Systemic therapy

    Gemcitabine, cisplatin and durvalumab lead first-line care; molecular profiling opens targeted options like pemigatinib and ivosidenib.

Why this guide matters

A rare cancer, a rapidly changing field.

Diagnosis, staging and treatment have all shifted meaningfully in the last few years. These three points shape everything else on this page.

  • Specialist HPB centres matter

    Care at a specialist-commissioned unit - King’s, Royal Free, Freeman, Birmingham or Manchester - is associated with better outcomes at every stage.

  • Molecular profiling opens doors

    FGFR2 fusions, IDH1 mutations, NTRK fusions and HER2 changes can each unlock a targeted therapy - ask whether your tumour has been profiled.

  • Immunotherapy has arrived

    Durvalumab added to gemcitabine and cisplatin is now standard first-line care for advanced biliary-tract cancer, based on the TOPAZ-1 trial.

How the diagnosis is made

From first jaundice to a clear plan.

The steps a UK hepatobiliary team will normally follow, in order - so you know what to expect and why each is done.

  1. 01

    Assessing

    History and risk factors

    A careful look at PSC, liver flukes, chronic biliary inflammation, congenital biliary anomalies, hepatitis B or C, cirrhosis, obesity and smoking.

  2. 02

    Assessing

    Bloods and tumour markers

    Obstructive liver function tests with a raised bilirubin, alkaline phosphatase and GGT, plus CA 19-9 and CEA to help characterise the picture.

  3. 03

    Assessing

    MRI and MRCP

    The gold-standard non-invasive test - maps the biliary tree, defines tumour extent and vascular involvement without a contrast injection into the ducts.

  4. 04

    Confirming

    CT chest, abdomen and pelvis

    Staging cross-section to look at nodes, vascular anatomy and distant disease - specialist-commissioned as part of the HPB pathway.

  5. 05

    Confirming

    ERCP with brush cytology

    Endoscopic sampling of the stricture, often combined with cholangioscopy (SpyGlass) for targeted biopsies - specialist-commissioned.

  6. 06

    Confirming

    Endoscopic ultrasound

    Refines local staging, samples suspicious nodes and helps decide between resection, transplant assessment and palliative pathways.

  7. 07

    Preparing

    Specialist HPB MDT review

    Discussion at a specialist-commissioned HPB centre such as King’s, Royal Free, Freeman, Birmingham or Manchester to agree the definitive plan.

Typical timeline: a first specialist visit to a definitive MDT plan in a few short weeks.

Symptoms

What hilar cholangiocarcinoma actually looks like.

Painless jaundice is the hallmark - alongside itch, weight loss and, sometimes, cholangitis. The features below are the ones that most often bring people to a doctor.

  • Painless jaundice

    Yellow skin and eyes, dark urine and pale stools - typically without pain, and the most common presenting sign.

  • Pruritus

    Persistent itching, often severe at night, driven by bile-salt accumulation as the ducts obstruct.

  • Weight loss and fatigue

    Unintentional weight loss with reduced appetite and low energy, often over weeks rather than months.

  • Right upper quadrant pain

    A dull ache under the right ribs - usually late, and worth taking seriously in anyone with new jaundice.

  • Cholangitis

    Fevers, rigors and jaundice from infected obstructed bile - an emergency needing urgent drainage and antibiotics.

  • Hepatomegaly

    A palpably enlarged liver as ducts back up - sometimes with a tender edge or right-sided fullness.

  • Steatorrhoea

    Pale, greasy, floating stools from impaired bile flow into the gut - often noticed alongside pale-clay stools.

  • Red flag - painless jaundice

    Any adult with painless obstructive jaundice needs urgent imaging - hilar cholangiocarcinoma is high on the differential.

Treatment

How hilar cholangiocarcinoma is treated in the UK.

Curative surgery or transplant where possible, biliary drainage for symptom control, and modern systemic and liver-directed options for advanced disease.

  • Major hepatectomy and bile-duct resection

    The main curative operation - liver resection with bile-duct excision and a Roux-en-Y hepaticojejunostomy, in specialist-commissioned HPB centres.

  • Neoadjuvant chemoradiotherapy and liver transplant

    For selected unresectable disease under the Mayo protocol - assessed through a specialist liver transplant clinic. See /treatments/liver-transplant-clinic/.

  • Biliary drainage

    Percutaneous or endoscopic stenting to relieve jaundice, cholangitis and pruritus - often the first therapeutic step before any oncological plan.

  • Gemcitabine and cisplatin

    The first-line systemic backbone based on the ABC-02 trial - the standard chemotherapy platform for advanced biliary-tract cancer.

  • FOLFOX (second-line)

    Oxaliplatin-based chemotherapy after progression - supported by the ABC-06 trial as a modest but meaningful second-line option.

  • Durvalumab plus chemotherapy

    Immunotherapy added to gemcitabine and cisplatin, based on TOPAZ-1 - approved in 2022 and specialist-commissioned. See /treatments/durvalumab-cholangiocarcinoma-clinic/.

  • Targeted therapy - FGFR2 and IDH1

    Pemigatinib, infigratinib or futibatinib for FGFR2 fusions; ivosidenib for IDH1-mutated disease - specialist-commissioned with molecular profiling.

  • Liver-directed therapy

    Y-90 radioembolisation, selective TACE, radiofrequency ablation and stereotactic body radiotherapy - specialist-commissioned in selected cases. See /treatments/tare-y90-clinic/.

What this guide is based on

The sources behind every claim on this page.

UK national guidance and specialist society standards, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your hepatobiliary team knows your history and imaging and can tell you which parts of this page apply to you. If in doubt, ask them.

  • British Society of Gastroenterology (BSG). Guidelines on the diagnosis and management of cholangiocarcinoma.

  • NICE. Guidance on biliary-tract cancers and targeted therapies (pemigatinib, ivosidenib, durvalumab).

  • International Liver Cancer Association (ILCA). Consensus statement on intrahepatic and perihilar cholangiocarcinoma.

  • MHRA and EMA. Product information for durvalumab, pemigatinib, ivosidenib, infigratinib and futibatinib.

Red flags

When to seek urgent help.

Most of the pathway is managed electively at a specialist centre. These are the situations that need urgent attention along the way.

  • Acute cholangitis

    Fever, rigors and jaundice mean infected obstructed bile - emergency admission for antibiotics and urgent biliary drainage.

  • Rapidly rising bilirubin

    A sharp climb in bilirubin with worsening pruritus needs prompt HPB review - stents or PTC drainage may be required.

  • Weight loss with new jaundice

    Unintentional weight loss alongside painless jaundice warrants a two-week-wait pathway and specialist imaging.

  • Cirrhosis with new biliary changes

    New biliary strictures on a background of PSC or cirrhosis need HPB assessment - see /conditions/cirrhosis/.

  • PSC surveillance findings

    Any dominant stricture, rising CA 19-9 or new mass in known PSC deserves urgent specialist evaluation.

  • Post-ERCP deterioration

    Fever, worsening pain or falling blood pressure after ERCP is an emergency - return to the hospital promptly.

  • Encephalopathy

    Confusion, drowsiness or reversed sleep-wake pattern in advanced disease points to hepatic decompensation - see /conditions/hepatic-encephalopathy/.

  • GI bleeding

    Vomiting blood or passing melaena in the context of a known biliary cancer needs immediate hospital assessment.

  • Unresolved pruritus

    Severe itch that fails to settle after drainage may need medication changes and a review of stent function.

Living with it

A demanding diagnosis, with a clear support structure.

Four things make the biggest difference across the whole journey - the team, the tumour profile, symptom control and early palliative support.

A quiet reminder

Ask what has been ruled in - and what is being watched.

Understanding what has been decided and what is still open makes appointments calmer and decisions clearer.

  1. 01 Team

    Stay under a specialist HPB team

    Care is best delivered at a specialist-commissioned centre - continuity with the same MDT matters through every phase.

  2. 02 Profile

    Molecular profiling is worth it

    FGFR2, IDH1, NTRK and HER2 findings can open targeted options - ask whether tumour molecular profiling has been done.

  3. 03 Symptoms

    Manage jaundice and itch early

    Drainage, cholestyramine and dose-appropriate antihistamines can transform day-to-day comfort - flag itch early.

  4. 04 Support

    Bring in palliative care sooner

    Early palliative-care involvement improves symptom control, quality of life and family support - alongside active treatment, not instead of it.

Frequently asked

Everything we get asked about hilar cholangiocarcinoma.

Quick answers on diagnosis, curative options, systemic therapy and liver-directed treatment.

  • What is hilar cholangiocarcinoma?

    A cancer arising at the confluence of the right and left hepatic ducts - historically called a Klatskin tumour. It is the most common form of cholangiocarcinoma and typically presents with painless obstructive jaundice.

  • What causes it?

    Most cases are sporadic, but recognised risk factors include primary sclerosing cholangitis (PSC), liver flukes, chronic biliary inflammation, congenital biliary anomalies (Caroli disease and choledochal cysts), hepatitis B or C, cirrhosis, obesity and smoking.

  • How is it diagnosed?

    The pathway usually starts with obstructive liver function tests and tumour markers (CA 19-9, CEA), followed by MRI and MRCP as the gold-standard imaging, CT for staging, and ERCP with brush cytology or cholangioscopy for tissue. Endoscopic ultrasound refines local staging before a specialist HPB MDT decides the plan.

  • Is it curable?

    Cure is possible with major hepatectomy and bile-duct resection with Roux-en-Y hepaticojejunostomy in selected patients. For carefully chosen unresectable cases, neoadjuvant chemoradiotherapy followed by liver transplant under the Mayo protocol offers another curative route.

  • What if the tumour cannot be removed?

    First-line treatment is usually gemcitabine and cisplatin plus durvalumab, based on the TOPAZ-1 trial. Molecular profiling can identify FGFR2 fusions (pemigatinib, infigratinib, futibatinib), IDH1 mutations (ivosidenib), NTRK fusions and HER2 changes that open targeted or antibody-based options.

  • What about liver-directed treatments?

    Y-90 radioembolisation, selective TACE, radiofrequency ablation and stereotactic body radiotherapy have a role in carefully selected patients. These are specialist-commissioned decisions taken at the HPB MDT alongside systemic therapy.

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