Mucin producing adenocarcinomas that arise from bile ducts


Mucin producing adenocarcinomas that arise from bile ducts

Types: intrahepatic, hilar or central (65%) and peripheral (30%)

Nodular tumors arising at the bifurcation of the common bile duct are called Klatskin tumors and are often associated with a collapsed gallbladder

Predisposing factors

Primary sclerosing cholangitis


Fibropolycystic liver disease

Caroli disease (bile duct ectasia)

Alcoholic liver disease

Choledochal cyst


HBV, HCV infection

Thorotrast exposure(formerly used in radiography of biliary tree)

Opisthorchis viverrini and Clonorchis sinensis infestation

Premalignant lesions

Biliary intraepithelial neoplasia (BiIIN 1, 2,3)

Mucinous Cystic Neoplasm

Intraductal Papillary Biliary Neoplasia

Clinical features, diagnosis and treatment

Typically presents with painless jaundice often with pruritis and weight loss

CEA, CA 19-9, and CA-125 are often elevated in CCC patients and are useful for following response to therapy