Primary Jejunal Diffuse Large B-Cell Lymphoma with Multiple Skip Ulcers and Perforation Mimicking Crohn's Disease

ABSTRACT

Most primary intestinal lymphomas are single exophytic or annular tumor masses with diffuse large B-cell lymphoma (DLBCL) as the most common histological type. Crohn's disease is a chronic inflammatory bowel disease with multiple skip ulcers. Here we reported the case of an elderly male presented with hollow organ perforation. Segmental resection of the jejunum revealed 5 skip ulcers, grossly mimicking Crohn’s disease. Pathological examination revealed diffuse sheets of large neoplastic cells expressing CD20, bcl-2, bcl-6 and MUM1 but not CD3 or CD10, a DLBCL of nongerminal center B-cell phenotype. In situ hybridization for Epstein-Barr virus was negative. Locus-specific interphase fluorescence in situ hybridization assay showed negative results for chromosomal aberrations at the IGH, BCL2, BCL6 and MYC loci. The clinical staging was IE and the patient received rituximab and combination chemotherapy. He was in complete remission for 14 months but unfortunately succumbed to an unidentified central nervous system infection 4 months later without evidence of tumor recurrence. Although multiple skip ulcers mimicked Crohn's disease, they were transverse in direction as opposed to the usual longitudinal ulcers in Crohn's disease. The correct diagnosis was reached by careful histopathological examination and ancillary studies.

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