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
Fever, rigors, hypotension or confusion with malignant jaundice indicates cholangitis and requires emergency sepsis treatment plus appropriately planned biliary decompression.
Recurrent jaundice, pale stool, fever or pain after palliation suggests occlusion or migration; BSG advises cross-sectional reassessment before repeat endotherapy.
Investigation priorities
Stage every suspected CCA, define primary extent and vascular relations, and identify nodal or distant spread.
Management branches
Imaging, jaundice or a dominant stricture raises a credible suspicion of cholangiocarcinoma without emergency sepsis.
- Classify the likely site, review benign alternatives and send liver, renal, clotting and inflammatory blood tests with a careful biliary history.
- Obtain multiphasic CT of chest, abdomen and pelvis, adding contrast MRI and MRCP for proximal or intrahepatic anatomy before endoscopy.
- Route the complete dataset rapidly to the regional HPB MDT, which defines resectability, drainage objectives and the safest tissue route.
- Explain uncertainty honestly: negative cytology may not exclude cancer, and surgery can occasionally provide both cure and definitive diagnosis.