Primary extrahepatic bile duct lymphoma mimicking Klatskin’s tumor, dramatic response to chemotherapy

INTRODUCTION: Non- Hodgkin’s lymphoma (NHL) mimicking Klatskin’s tumor is rare but possible. It can be confused with tumors for which major surgery is needed. Imaging modalities have similar features in Klatskin’s tumor and non-Hodgkin’s lymphoma. PRESENTATION OF CASE: We herein report a patient who was first thought having a Klatskin’s tumor and prepared for major surgery. A month later, obstructive jaundice developed and a second magnetic resonance-magnetic resonance cholangiopancreotography showed a doubling size of the tumor in one month. In terms of tumor behavior, lymphoma was suspected and a tru-cut biopsy was performed by interventional radiology. Pathological examinations revealed non-Hodgkin’s lymphoma large B-cell type. After 4 cycles of chemotherapy by hematology-oncology department, complete cure was achieved. DISCUSSION: Since the radiologic and clinical features are confusable in Klatskin’s tumor and non-Hodgkin lymphoma. Tumor biologic behavior and other biochemical parameters together may put a suspicion in mind. Upon suspicion biopsy must be done radiologically. CONCLUSION: Primary liver lymphoma arising from the bile ducts is extremely rare and radiologic imaging features do not differ from cholangiocarcinoma. A strong suspicion may prevent unnecessary surgery. Since the treatment of non-Hodgkin’s lymphoma is mainly chemotherapy.