Gastrointestinal mantle cell lymphoma (MCL) is an aggressive subtype of B-cell lymphoma accounting for 1 % to 2 % of non-Hodgkin lymphomas. Patients usually present with lymphadenopathy, bone marrow, and extranodal involvement. The commonest gastrointestinal (GI) manifestation is multiple lymphomatous polyposis, in which multiple lymphoid polyps are present in the GI tract [1, 2]. The ileocecal region is the commonest site involved. GI symptoms include pain, obstruction, diarrhea, or hematochezia. Multiple lymphomatous polyposis may also be seen in marginal B cell lymphomas, mucosa associated lymphoid tissue (MALT), and follicular lymphomas. Despite prompt remission, prognosis remains poor in view of increased relapse rates. Median survival is 3 to 5 years. Our patient presented with bloody diarrhea for 6 weeks. Colonoscopy revealed diffuse submucosal polypoidal lesions (Fig. 1, Supplemental Fig. S1) and upper GI endoscopy revealed similar multiple polypoidal lesions in the stomach (Supplemental Fig. S2) and duodenum. CT scan abdomen (Supplemental Fig. S3a, b) revealed nodular lesions involving the entire GI tract with intraabdominal lymphadenopathy. Biopsies from the nodules revealed malignant B-cell lymphoma with immunohistochemistry positive for CD20, CD5, and cyclin D1 (Supplemental Fig. S4.) He received rituximab-based chemotherapy which resulted in significant tumor regression (Supplemental Fig. S5) and prompt symptomatic palliation.
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Triozzi PL, Borowitz MJ, Gockerman JP. Gastrointestinal involvement and multiple lymphomatous polyposis in mantle zone lymphoma. J Clin Oncol. 1986;4:866–73.
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Fig. S1
Multiple polypoid lesions were seen on the cecal pole and ileo-cecal valve (JPEG 169 kb)
Fig. S2
Polypoidal lesions involving the body of the stomach (JPEG 151 kb)
Fig. S3
CT abdomen demonstrating diffuse polypoidal involvement of the stomach wall (a) and a large polypoidal lesion in the terminal ileum (b) (JPEG 948 kb)
Fig. S4
Immunohistochemistry stained strongly for cyclin D1 marker (JPEG 2190 kb)
Fig. S5
Complete regression of the lymphomatous lesions noted after 2 weeks of intensive chemotherapy (JPEG 1200 kb)
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Aiman, S., Chakrapani, A., Sawaimoon, S. et al. Multiple lymphomatous polyposis: Characteristic endoscopic features. Indian J Gastroenterol 34, 87 (2015). https://doi.org/10.1007/s12664-014-0497-4
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DOI: https://doi.org/10.1007/s12664-014-0497-4