A Klatskin tumor, also known as Hilar Cholangiocarcinoma, is a cholangiocarcinoma (cancer of the biliary tree) occurring at the confluence of the right and left hepatic bile ducts.
Diagnosis is suspected on clinical and laboratory findings. Serum carbohydrate antigen (CA) 19-9 is a glycoprotein tumor marker found to be elevated in most cases of Klatskin tumors. Increased levels of alkaline phosphate, conjugated bilirubin and gamma-glutamyl transpeptidase are also noted. Abdominal imaging, visualization of the biliary tree and biopsies of the lesion are necessary to make the diagnosis. Endosonography (EUS) guided fine needle aspiration (FNA) of hilar lymph nodes is the most useful tool in the diagnosis and staging of Klatskin tumors. Brush cytology and percutaneous biopsies have a low sensitivity for diagnosis. Ultrasound, and contrast enhanced helical computerized computed tomography (CT) can be used in visualizing the extent of disease.
Autoimmune cholangitis and primary biliary non-Hodgkin's lymphoma are differential diagnoses of Klatskin tumors.
Prognosis remains poor today.
Because of their location these tumours present late and therefore are usually not resectable at the time of presentation. Complete resection of the tumour offers hope of long term survival and of late there has been renewed interest in liver transplantation from deceased donors along with adjuvant therapy.