Case Report | Open Access
Extranodal NK/T-Cell Lymphoma without Nasal Cavity Involvement Associated with Epstein-Barr Virus: A Multimodality-Based Diagnosis of Two Cases
We report two cases of extranodal NK/T-cell lymphoma, nasal type, in immunocompetent patients without nasal cavity involvement. In the two cases, the initial presumptive diagnosis was tuberculosis and there was a rapid dissemination of the tumor with short survival after the hospital admittance. An autopsy was performed showing infiltration in several organs including lymph nodes and mesenteric and retroperitoneal fat. Histological sections showed an angiocentric and angiodestructive growth pattern and the immunophenotype was CD45+, CD3+ (cytoplasmic), as well as Granzyme B+ and EBV+. However, CD56 expression was only positive in a case in which the molecular study showed T-cell gene rearrangement with monoclonal appearance and associated with hemophagocytic syndrome. These cases represent rare examples of NK/T-cell lymphoma disseminated outside the nasal cavity highly aggressive that lead to the rapid death of the patients.
- J. K. C. Chan, E. S. Jaffe, and E. Ralfkiaer, “Extranodal NK/T-cell lymphoma, nasal type,” in Pathology and Genetics: Tumours of Haematopoietic and Lymphoid Tissues, E. S. Jaffe, N. L. Harris, H. Stein, and J. W. Vardiman, Eds., World Health Organization Classification of Tumours, pp. 204–207, IARC Press, Lyon, France, 2001.
- F. Aydin, P. M. Bartholomew, and D. G. Vinson, “Primary T-cell lymphoma of the brain in a patient at advanced stage of acquired immunodeficiency syndrome,” Archives of Pathology and Laboratory Medicine, vol. 122, no. 4, pp. 361–365, 1998.
- F. Cobo, P. Talavera, H. Busquier, and Á. Concha, “CNK/T-cell brain lymphoma associated with Epstein-Barr virus in a patient with AIDS,” Neuropathology, vol. 27, no. 4, pp. 396–402, 2007.
- L. S. Young and A. B. Rickinson, “Epstein-Barr virus: 40 years on,” Nature Reviews Cancer, vol. 4, no. 10, pp. 757–768, 2004.
- P. Porcu, R. A. Baiocchi, and C. Magro, “Recent developments in the biology and therapy of T-cell and natural killer-cell lymphomas,” Current Opinion in Oncology, vol. 15, no. 5, pp. 353–362, 2003.
- J. P. Greer, M. C. Kinney, and T. P. Loughran, Jr., “T cell and NK cell lymphoproliferative disorders,” Hematology, vol. 2001, no. 1, pp. 259–281, 2001.
- Y. L. Kwong, A. C. L. Chan, R. Liang et al., “ NK lymphomas: clinicopathological features and prognosis,” British Journal of Haematology, vol. 97, no. 4, pp. 821–829, 1997.
- M. M. C. Cheung, J. K. C. Chan, W. H. Lau et al., “Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients,” Journal of Clinical Oncology, vol. 16, no. 1, pp. 70–77, 1998.
- S. B. Ng, K. W. Lai, S. Murugaya et al., “Nasal-type extranodal natural killer/T-cell lympholmas: a cliniclopathologic and genotypic study of 42 cases in Singapore,” Modern Pathology, vol. 17, no. 9, pp. 1097–1107, 2004.
Copyright © 2009 Fernando Cobo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.