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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Cholangiocarcinoma

Gastroenterology and hepatology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

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Escalate

Fever, rigors, hypotension or confusion with malignant jaundice indicates acute cholangitis. Begin emergency sepsis treatment and coordinate prompt biliary decompression with the hepatopancreatobiliary team. Recurrent jaundice, pale stool, fever or pain after stenting suggests stent occlusion or migration and requires urgent reassessment.

Synopsis

Classify suspected cholangiocarcinoma by anatomical site, protect staging and tissue strategy, manage obstruction safely and route treatment through a specialist hepatobiliary multidisciplinary team.

  • Classify cholangiocarcinoma as intrahepatic, perihilar or distal because symptoms, imaging, tissue route, drainage, surgery and molecular patterns differ materially by site.
  • Extrahepatic disease commonly presents with progressive jaundice, dark urine, pale stool and pruritus; intrahepatic tumours may present as a liver mass, pain, weight loss or incidental imaging abnormality.
  • Primary sclerosing cholangitis, choledochal cysts and selected chronic biliary inflammatory disorders increase risk, but most patients do not have an obvious pre-existing syndrome.
  • BSG recommends multiphasic contrast CT of chest, abdomen and pelvis for all types; add contrast MRI and MRCP for perihilar and intrahepatic tumours to define biliary extent and satellites.
  • Obtain staging imaging before endoscopic intervention when the patient is stable, because stents and inflammation can obscure anatomy; cholangitis or organ-threatening obstruction may require immediate drainage instead.
  • CA19-9 cannot diagnose cholangiocarcinoma: cholestasis and infection can elevate it, and people lacking Lewis antigen may not produce it despite cancer.
  • Tissue strategy is site- and intent-dependent; BSG requires MDT planning, and perihilar endotherapy should not proceed before discussion at an HPB treatment centre.
  • R0 surgical resection is the only established curative treatment; advanced disease should receive early molecular profiling, specialist systemic-therapy review and palliative support alongside active oncology.

Key red flags

Acute infected obstruction

Fever, rigors, hypotension or confusion with malignant jaundice indicates cholangitis and requires emergency sepsis treatment plus appropriately planned biliary decompression.

Stent dysfunction

Recurrent jaundice, pale stool, fever or pain after palliation suggests occlusion or migration; BSG advises cross-sectional reassessment before repeat endotherapy.

Investigation priorities

01
Multiphasic contrast CT chest, abdomen and pelvisFirst step

Stage every suspected CCA, define primary extent and vascular relations, and identify nodal or distant spread.

Management branches

DiagnosisProtected staging sequence

Imaging, jaundice or a dominant stricture raises a credible suspicion of cholangiocarcinoma without emergency sepsis.

  1. Classify the likely site, review benign alternatives and send liver, renal, clotting and inflammatory blood tests with a careful biliary history.
  2. Obtain multiphasic CT of chest, abdomen and pelvis, adding contrast MRI and MRCP for proximal or intrahepatic anatomy before endoscopy.
  3. Route the complete dataset rapidly to the regional HPB MDT, which defines resectability, drainage objectives and the safest tissue route.
  4. Explain uncertainty honestly: negative cytology may not exclude cancer, and surgery can occasionally provide both cure and definitive diagnosis.

Key medicines

Capecitabine after resectionUse only the current specialist adjuvant protocol, typically delivered over a defined course with body-surface-area calculation and cycle review.Check DPD status under current policy, renal and liver function, diarrhoea, mucositis, hand-foot toxicity, cardiac risk and interacting anticoagulants.
First-line advanced biliary cancer regimenDeliver a nationally commissioned oncology protocol such as gemcitabine-platinum with any approved immunotherapy component, using current cycle-specific prescribing.Approvals evolve; monitor marrow, renal, hepatic, hearing, infection and immune-mediated toxicity and adapt to biliary sepsis or declining performance.
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • BSG cholangiocarcinoma guideline hub
  • BSG cholangiocarcinoma full guideline
  • NICE NG12 suspected cancer recommendations
  • BSG ERCP minimum service standards

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom