History and admission findings: A 53-year-old man with jaundice was referred with suspected cholangiocarcinoma of the porta hepatis. He was found to be in a reduced general and nutritional condition. Cholinesterase was decreased, total bilirubin, GOT, GPT and alkaline phosphatase were increased.
Investigations: Abdominal contrast-enhanced computed tomography revealed a centrally located and contrast-enhancing mass at the proximal common hepatic duct. Metastases were not detectable. Endoscopic retrograde cholangiography showed a stenosis of the proximal common hepatic duct. An additional magnetic resonance tomography with cholangio-pancreatography confirmed the centrally located tumor as well as the stenosis of the common hepatic duct.
Diagnosis, treatment and course: The diagnostic tests were suggestive of a stage IV cholangiocarcinoma of the porta hepatis (Klatskin's tumor). The origin was subsequently proven histologically. There was no evidence of intra- or extrahepatic metastases. An interventional embolization of the right portal vein was performed preoperatively to stimulate the growth of the left hepatic lobe. But before surgery could take place a percutaneous transhepatic cholangiography and biliary drainage had to be done followed by an extended right-sided hemihepatectomy. During a 12 month follow-up no recurrence was observed.
Conclusion: Klatskin's tumor is rare and requires interdisciplinary collaboration. Recent developments in diagnostic, interventional and surgical methods result in a better prognosis.