[Cholangiolar carcinoma]

Cholangiocarcinoma affects both sexes equally, is more prevalent in individuals age 50 to 70. Associations with many other diseases are known, in particular with primary sclerosing cholangitis, ulcerative colitis and parasitic infestation (Clonorchis sinensis, Opisthorchis viverrini). About 95% are histopathologically classified as adenocarcinoma. The localisation of the tumor determines clinical course and prognosis. If the tumor is located above the hepatic duct bifurcation, only one side of the biliary tree may be obstructed, resulting in atrophy of the corresponding liver lobe, without clinical manifestation of jaundice. Obstructive jaundice is the characteristic symptom of hilar tumor localisation. Laboratory examinations show hyperbilirubinemia and liver enzymes indicating cholestatis are elevated. Pathological levels of the tumor markers carcinoembryonic antigen (CEA) and CA 19-9 are frequently found. Ultrasonography in combination with endoscopic retrograde or percutaneous transhepatic cholangiography and cytological examination of aspirates are of main diagnostic importance. Treatment consists of curative resection or palliative decompression to relieve jaundice, the latter can be performed using endoscopic implantable self-expanding metal stents. Cytotoxic chemotherapy or liver transplantation show no satisfactory results. The prognosis is mainly poor, only few patients survive more than 6 months after diagnosis.