Duodenal mucosa-associated lymphoid tissue lymphoma: treatment with oral cyclophosphamide

Small cell mucosa-associated lymphoid tissue (MALT) lymphomas rarely affect the duodenum, and optimal treatment has not been defined. The aim of this case series was to determine the clinical features and outcome of duodenal MALT lymphoma in four patients (three men, one woman; median age 52 yr) treated with cyclophosphamide p.o. Initial manifestations were abdominal pain (n = 4), vomiting (n = 2), and an obstructive syndrome (n = 1). MALT lymphoma was diagnosed on the basis of endoscopic biopsies. It was localized in the duodenum in three cases and involved the entire small bowel in one case. Tumor infiltration was limited to the duodenal wall in one case and was associated with locoregional lymphadenopathy in three cases. The patients were graded EI (n = 1) and EII1 (n = 3), respectively, according to the Ann Arbor classification revised by Musshof. Cyclophosphamide, 100 mg daily, was administered p.o. for 18 months. Gastroscopy with biopsies, radiography of the small intestine and abdominal CT (CT) were performed every 6 months. Complete remission was defined by morphological and histological normalization, and partial remission as morphological normalization only. Follow-up lasted from 9 to 65 months. Three patients were in complete remission at 18 months: two relapsed after 2 yr and one was still in complete remission at 65 months. The patient with 9 months of follow-up was in complete remission at 6 months. The two patients who relapsed did not complain of symptoms, and no morphological abnormalities were seen. Relapse was diagnosed on histological grounds. Cyclophosphamide monotherapy p.o. thus seems well adapted to this slowly progressive disease, but it is unclear whether it should be resumed in the case of histological relapse or only in the case of symptomatic relapse. (Am J Gastroenterol 2000;95:536-539. (O 2000 by Am. Coll. of Gastroenterology)