Primary non-Hodgkin's lymphoma of the bile ducts mimicking cholangiocarcinoma.

BACKGROUND: Primary non-Hodgkin's lymphoma (NHL) of the liver and bile duct mimicking cholangiocarcinoma is rare.
METHODS: The clinical and radiologic features and the treatment of 2 patients with primary NHL of the bile ducts are presented and analyzed together with cases collected from a review of the English literature between 1966 and 2003.
RESULTS: Fifteen patients with primary NHL, including our 2 patients, presented with clinical features mimicking cholangiocarcinoma. All had jaundice; 9 had systemic symptoms; 7 had abdominal pain; and 5 had mass lesions. All had biliary strictures as shown on cholangiography. Two patients were infected with human immunodeficiency virus-1. In only 1 patient was the diagnosis established without surgery. Immunophenotyping in 10 patients showed 9 B-cell tumors and 1 T-cell tumor. Twelve patients underwent resection. Seven received chemotherapy immediately after the diagnosis was made. Only 3 patients have survived more than 3 years, with the longest survival being 68 months.
CONCLUSIONS: Non-Hodgkin's lymphoma of the liver and bile duct must be considered in the differential diagnosis of patients with obstructive jaundice. If the correct diagnosis is made before surgery, current protocols of chemotherapy may be the primary modality of therapy. Surgical resection should be reserved to address complications of biliary obstruction or the failure of chemotherapy to eradicate localized disease.