Primary CD56+ NK/T-cell lymphoma of the rectum accompanied with refractory ulcerative colitis

Abstract

A case of primary NK/T-cell lymphoma of the rectum accompanied with ulcerative colitis (UC) in a 73-year-old man is reported. He had a 6-year history of repeated admission to our hospital for UC. Total colonoscopy performed 4 months after resolution of refractory UC complicated by cytomegalovirus colitis showed a markedly submucosal tumor in the rectum, which was histologically diagnosed as malignant lymphoma. The findings of computed tomography of the chest and abdomen, gallium scintigraphy, abdominal ultrasonography, and upper gastrointestinal endoscopy showed no abnormal lesions. Therefore, based on a diagnosis of localized rectal lymphoma with UC, proctocolectomy was performed. The resected specimen showed three submucosal tumors in the rectum with local nodal involvement. Histologically, the tumors were characterized by diffusely infiltrating sheets of large atypical lymphoid cells, which were negative for CD4, CD8, and CD20 but were positive for CD56, CD3, and granzyme B. The presence of Epstein-Barr virus (EBV) infection in neoplastic cells was shown by in situ hybridization for EBV-encoded early small RNA1 (EBER-1). Based on these findings, the patient was diagnosed with primary CD56+ NK/T-cell lymphoma of the rectum (stage IIE). This is the first case report of primary rectal NK/T-cell lymphoma accompanied with UC.

EBV-associated Peripheral T-Cell Lymphoma of Gastrointestinal Tract Presented With Widespread Chronic Inflammation: A Case Report

“…Several single case reports of GI ENKTL as well as identified similar features including CD56 expression, necrosis and angioinvasion. [16][17][18] Although our case might be classified in this category, the clinical presentation, histologic morphology, and phenotypic features do not support this diagnosis. Our patient presented with a widespread GI inflammatory response before overt T-cell lymphoma developed.…”

Rectal MALT lymphoma associated with ulcerative colitis

“…About one-third to onehalf of reported colorectal lymphomas are MALT type, commonly affecting the cecum, with rectum being an unusual site. 8,9 Rarely, large T-cell, 10 mixed B cell and T cell 11 and NK/Tcell 12 lymphoma have also been reported. In a review of 34 cases of rectal MALT lymphoma none had underlying ulcerative colitis.…”

Primary Small Intestinal Natural Killer/T Cell Lymphoma Mimicking Tuberculous Peritonitis: Report of a Case and Review of the Literature

“…Gastric and colonic lymphomas can be diagnosed via conventional EGD and colonoscopy (1). However, NK/T-L derived from the small intestine, which has a low incidence, is mostly diagnosed incidentally or when the patient presents with acute abdomen.…”

“…They are almost always of the B-cell lineage. Natural killer/T-cell lymphomas (NK/T-Ls), especially those occurring in the GI tract, are rare; recently some cases have been described in a few reports (1,2). Tuberculous (TB) peritonitis is a subacute disease and its symptoms evolve over several weeks to a few months.…”