Sclerosing carcinoma of the right hepatic duct at the porta hepatis: anicteric presentation of early hilar cholangiocarcinoma.

: Asymptomatic presentation of hilar cholangiocarcinoma is exceptional. We report an asymptomatic patient presenting with an abnormal alkaline phosphatase who proved to have a unilateral hilar cholangiocarcinoma obstructing the right heptic duct at the porta hepatis. Hyperbilirubinemia was absent. Subsequent to partial tumor resection and palliative decompression with right intrahepatic cholangiojejunostomy, the patient developed cholangitis with abscess formation. The efficacy of biopsy, tumor resection, and surgical decompression in hilar cholangiocarcinoma is discussed. Postmortem histology revealed biliary cirrhosis and acute cholangitis in the obstructed right liver lobe and minimal portal fibrosis in the unobstructed left liver lobe. The absence of bile stasis suggests compensatory bile excretion mechanism in the unobstructed left lobe. We believe the clinical and pathological findings in the patient to be part of the early natural history of hilar cholangiocarcinoma. Complete workup of asymptomatic alkaline phosphatase elevations in patients without previous history of hepatobiliary disease may prove fruitful in revealing incipient localized, unilateral bifurcation tumors.