Lymphoma of the extrahepatic biliary ducts in acquired immunodeficiency syndrome

Gastrointestinal neoplasia in acquired immunodeficiency syndrome (AIDS) frequently presents as a non-Hodgkin's lymphoma or Kaposi's sarcoma, at sites including the oral cavity, esophagus, small bowel, and mesenteries. Primary lymphoma of the biliary ducts, even in the setting of AIDS, is rare. This patient, an older male with no preoperatively known AIDS risk factors, presented with painless jaundice and radiographic findings of focal hepatic duct stenosis, suggesting either a Klatskin tumor or sclerosing cholangitis. Only after the pathologic diagnosis of a malignant large cell lymphoma from the en bloc tumor specimen was his human immunodeficiency virus status ascertained and noted to be positive.