ABSTRACT
Primary gastrointestinal lymphomas originating from the mucosa-associated lymphoid tissue (MALT) have been reported with increasing frequency. The stomach is the most frequent site of MALT lymphoma, and a relationship with Helicobacter pylori infection has been studied. However, primary MALT lymphoma arising from the ampulla of Vater is extremely rare, and its relationship with Helicobacter pylori infection is still obscure. We report here a case of a 71-year-old man with marginal zone B cell lymphoma of MALT with large B cell lymphoma of the ampulla of Vater that was not associated with Helicobacter pylori. A pancreaticoduodenectomy was performed, and the pathologic examination of the resected specimen confirmed the diagnosis. The patient tolerated just one course of systemic chemotherapy, but has been doing well for 18 months after surgery.