Primary biliary B-cell lymphoma of the cystic duct causing obstructive jaundice

A distinctive case of primary biliary lymphoma occurring in the cystic duct causing extrinsic compression of the porta hepatis in a 48-year-old woman is presented. Imaging studies revealed stricture of the common hepatic duct with a 2.5-cm nonhomogeneous mass at the porta hepatis, mimicking a Klatskin tumor. Exploratory laparotomy revealed a mass in the gallbladder neck with extension into the cystic duct akin to Mirizzi's syndrome. A soft, tan-yellow 1.0-cm mass was removed from the cystic duct, and infiltrating atypical lymphocytic nodules were identified with a final diagnosis of an anaplastic variant of diffuse large B-cell lymphoma.