Histopathological examination of the resected ileal mass revealed T-cell lymphoma. Microscopically, medium to large sized cells with vesicular nuclei including marked nucleoli with large, colorless cytoplasm were seen. No signs of celiac disease were found in the adjacent mucosa. The tumor cells were immunuhistochemically positive with CD45, CD3, CD4, CD8, CD56 and negative with pan-cytokeratin (panCK), CD20, CD79a, CD5 and CD30 (Figure 2A-D). After a careful anamnesis, no history or association with celiac disease was determined and the endomysial antibody and antigliadin antibody IgM and IgG tests were negative. Ebstein-Barr virus (EBV) antibodies IgM and IgG were positive for EBV viral capsid antigen (VCA) and EBV virus nuclear antigen (EBNA). However, no additional tumor was detected by screening in the nasopharynx, skin and soft tissues or other parts of the gastrointestinal tract. Further, immunohistochemically, anti-EBV latent membrane protein (anti EBV-LMP; Daco, Denmark) was also negative. Hence, the histopathological diagnosis of the tumoral mass was NK/T-cell lymphoma, and the patient was referred to medical oncology where he was placed on a Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) chemotherapy regimen. At the end of the six-month follow-up, the patient is now symptomless and is coming for routine check- ups.
The nasal type of the extranodal NK/T-cell lymphoma occurs usually in the nasopharyngeal region; it is CD56 positive CD8 negative, lacks usually T cell receptor (TCR) gene rearrangement, and is strongly associated with EBV[1,25-27]. It is described as ‘’NK/T ‘’rather than ‘’NK’’ because of some reported cases in which cytotoxic T-cell phenotype was observed[1,14]. Although, the present case had positive IgM and IgG antibodies for EBV VCA and EBNA, a diagnosis of a nasal type NK cell lymphoma is eliminated due to absence of nasopharyngeal involvement by the tumor. Additionally, the anti EBV-LMP in the tumor cells was negative. Thus, our case was diagnosed as NK/Tcell lymphoma.
NK/T-cell lymphoma of the terminal ileum is a rare entity that has similar endoscopic features of inflammatory bowel disease, and often invades serosa. Patients usually present at an advanced stage with peritonitis as a result of tumor perforation[24]. NK/T-cell lymphomas are reported in a few studies as case series and the true incidence is probably more frequent than expected[26,27]. Furthermore, NK/Tcell lymphoma of the terminal ileum can mimic Behçet’s enteritis or intestinal tuberculosis and should be considered in the differential diagnosis of these diseases. The true diagnosis can only be established by histopathological evaluation[28,29].
In conclusion, primary intestinal cytotoxic natural killerlike T-cell lymphoma is a rare entity, which is difficult to distinguish from other T-cell lymphomas. In addition to microscopic evaluation immunohistochemical analysis and serological tests are needed to reach a definitive diagnosis.
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