Mucosa-associated lymphoid tissue (
MALT) lymphoma of the papilla of Vater is rare, and little is known of either its association with Helicobacter pylori
infection or the optimal treatment modalities. We describe the first case of
MALT lymphoma involving the major papilla that remained unchanged despite eradication of H. pylori, but which regressed following
radiotherapy. A 46-year-old asymptomatic man was admitted to hospital for treatment of
MALT lymphoma involving the papilla of Vater. Duodenal endoscopy showed multiple granules around the major ampulla, and biopsies revealed mucosal proliferation of centrocyte-like cells, lymphoepithelial lesions, hyperplastic lymphoid follicles and plasmacytic differentiation. The
lymphoma cells were positive for B-cell but negative for T-cell markers, and expressed Bcl-2 but showed no immunoreactivity for CD5, CD10 and
cyclin D, consistent with
MALT lymphoma. The patient was successfully treated with triple
therapy of
lansoprazole,
amoxicillin and
clarithromycin for 1 week for coexisting H. pylori
infection in the stomach, but the
lymphoma lesions remained unchanged. Then, involved-field irradiation was applied at a total dose of 30 Gy delivered in 1.5 Gy fractions without any adverse events. Six months later, repeat endoscopy revealed disappearance of the granular lesions and lack of
lymphoma cells in biopsy specimens. Four years after the commencement of
radiotherapy, the patient is still
in complete remission.
Radiotherapy seems a safe and effective treatment modality for low-grade
MALT lymphoma of the ampulla of Vater.