Hilar cholangiocarcinoma (Klatskin tumour) arises at or near the confluence of the right and left hepatic ducts. Because the lesion obstructs the central bile ducts early, symptoms are usually biliary and appear while the tumour is still relatively small.

1. Painless jaundice
   Conjugated hyper-bilirubinaemia develops insidiously, producing progressive yellowing of skin and sclera, dark urine and clay-coloured stools; pruritus is often intense and may precede visible icterus.
2. Right-upper-quadrant discomfort
   A dull, non-colicky ache or sensation of fullness is common; pain is usually mild and does not parallel the degree of jaundice.
3. Cholangitis episodes
   Intermittent fever with rigors, right-sided tenderness and elevated C-reactive protein occur when malignant obstruction becomes infected, fulfilling Charcot triad.
4. Systemic features
   Fatigue, early satiety, unintentional weight loss and low-grade night sweats reflect chronic cholestasis and tumour cachexia.
5. Palpable gallbladder (Courvoisier sign)
   A distended, non-tender gallbladder may be felt below the right costal margin if the cystic duct remains patent—more frequent in distal tumours but occasionally present in hilar lesions.
6. Hepatomegaly and lobar atrophy–hypertrophy complex
   The obstructed lobe atrophies while the contralateral lobe compensates, leading to a firm, nodular liver edge and visible abdominal asymmetry.
7. Laboratory cholestasis
   Serum alkaline phosphatase and γ-glutamyl transferase are elevated in > 90 % of cases; total bilirubin climbs steadily as ductal involvement extends.
8. Advanced disease indicators
   Persistent fever, ascites, palpable left-supraclavicular node or rapidly rising bilirubin suggest unresectable disease or distant spread.