Colon MALT Lymphoma in a Patient with Multiple Endocrine Neoplasia Type I

Abstract

Purpose: Mucosa-associated lymphoid tissue (MALT) lymphoma is a rare cause of colon tumors. We describe MALT lymphoma arising in a patient with a history of multiple endocrine neoplasia type I (MEN-1). Methods: A 72-year-old man with MEN-1 syndrome presented for endoscopic work up of iron deficiency anemia. He was in his usual state of health, without abdominal pain, overt gastrointestinal bleeding or obstructive symptoms. Significant history included Zollinger-Ellison syndrome with resection of pancreatic gastrinoma (1994), duodenal carcinoid resection (1995), and hyperparathyroidism with partial parathyroidectomies (1985, 2002). Colonoscopy showed several large, ulcerated masses in the colon. Initial forceps biopsy specimens were inconclusive. A larger, snared specimen showed MALT-associated low-grade-B-cell-lymphoma. Positron emission tomography imaging showed only re-demonstration of malignant appearing colon masses. The patient underwent four weeks of rituximab and repeat imaging is in process to decide on maintenance therapy. Results: Chronic Helicobacter pylori (H. pylori) associated gastritis is a risk factor for gastric MALT lymphoma. In contrast, an H. pylori association in colon MALT lymphomas has not been clearly demonstrated. Furthermore, although there have been studies linking GI peptide over-expression with colon cancers, this has not specifically been reported in colon MALT lymphoma. Therefore, the contribution of this patient's underlying MEN-1 syndrome to the development of colon MALT lymphoma remains a question.Figure 1: MALT lymphoma.Figure 2: Significant invasion of mucosa glandular epithelium by lymphocytes, scattered large blast cells are seen.Conclusion: Here, we have described a case of primary colon MALT lymphoma associated with MEN-1 syndrome. This particular association has not been described previously in the literature.