A Unique Presentation of Primary Intestinal MALT Lymphoma as Multiple Lymphomatous Polyposis

Multiple lymphomatous polyposis is considered to be a rare condition, with most of the cases being extranodal counterpart of mantle cell lymphomas. We report a rare case of multiple lymphomatous polyposis of the gastrointestinal tract in which the patient presented with abdominal pain and bloody diarrhea. Computer tomography of the abdomen showed circumferential wall thickening with intramural mass involving caecum & ascending colon with enlarged pericolonic lymph nodes. The patient underwent right hemicolectomy. Immunohistologic findings were characteristic of MALT lymphoma. Microscopic examination of polypoidal masses and mesenteric lymph nodes revealed infiltration by pleomorphic, atypical lymphoid cells which were CD20 positive and negative for CD3, CD10, Cyclin D1. Lymphoepithelial lesions were also noted. Careful endoscopic evaluation and histopathological review along with an immunohistochemical panel is extremely useful for accurately diagnosing such cases and avoiding unnecessary surgery and inappropriate therapy.