We report a rare case of mucosa-associated lymphoid tissue (MALT) lymphoma of the common bile duct and the papilla of Vater. A 66-year-old man was admitted to our hospital with right upper quadrant pain and jaundice. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography showed common bile duct stenosis. Further endoscopy revealed swelling of the papilla of Vater, with erosion at the ileal end. The patient was pathologically diagnosed with MALT lymphoma and treated for Helicobacter pylori eradication, after which the lesions improved. However, 1 year later, the lesions began to enlarge again;thus, he was treated with 6 courses of combination chemotherapy (R-CHOP). After treatment, the tumor and erosion of the papilla of Vater disappeared and the stenosis of the common bile duct improved.
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