A rare case of primary non-Hodgkin's lymphoma of the main hepatic duct junction is reported. A 71-year-old man was admitted for treatment of obstructive jaundice. Radiological examination revealed stenosis of the main hepatic duct junction. Biliary drainage was not necessary because total bilirubin decreased spontaneously. A left hepatic and caudate lobectomy, combined with resection of bile ducts and lymph node dissection, was performed with the preoperative diagnosis of cholangiocarcinoma of the main hepatic duct junction. Macroscopic examination of the resected specimen revealed tumorous growth in the main hepatic duct junction. Histological and immunochemistry findings disclosed a mucosaassociated lymphoid tissue (MALT) lymphoma. The patient received three courses of combination chemotherapy [cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP)], and there was no evidence of recurrence 45 months after the surgery. Although primary non-Hodgkin's lymphoma of the main hepatic duct junction is extremely rare and difficult to diagnose preoperatively, aggressive surgery followed by chemotherapy, as here, is a possible curative option.
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