This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mraz-Gernhard, S. Articles by Kim, Y. H. Search for Related Content PubMed PubMed Citation Articles by Mraz-Gernhard, S. Articles by Kim, Y. H.

Natural Killer/Natural Killer-Like T-Cell Lymphoma, CD56+, Presenting in the Skin: An Increasingly Recognized Entity With an Aggressive Course

From the Departments of Dermatology, Pathology, and Radiation Oncology, Stanford University School of Medicine, Stanford; and Departments of Pathology and Dermatology, University of California, San Francisco, CA.

Address reprint requests to Serena Mraz-Gernhard, MD, Department of Dermatology, 900 Blake Wilbur Dr, W0069, Stanford, CA 94305; email: serenam{at}leland.stanford.edu

PURPOSE: To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin.

PATIENTS AND METHODS: This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters.

RESULTS: The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P = .0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect on survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P < .02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3, EBV, or multidrug resistance.

CONCLUSION: NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study. The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome.

This article has been cited by other articles:

				Y.-X. Li, H. Fang, Q.-F. Liu, J. Lu, S.-N. Qi, H. Wang, J. Jin, W.-H. Wang, Y.-P. Liu, Y.-W. Song, et al. Clinical features and treatment outcome of nasal-type NK/T-cell lymphoma of Waldeyer ring Blood, October 15, 2008; 112(8): 3057 - 3064. [Abstract] [Full Text] [PDF]

				C. Assaf, S. Gellrich, S. Whittaker, A. Robson, L. Cerroni, C. Massone, H. Kerl, C. Rose, A. Chott, S. Chimenti, et al. CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer J. Clin. Pathol., September 1, 2007; 60(9): 981 - 989. [Abstract] [Full Text] [PDF]

				L. Pagano, A. Gallamini, G. Trape, L. Fianchi, D. Mattei, G. Todeschini, A. Spadea, S. Cinieri, E. Iannitto, M. Martelli, et al. NK/T-cell lymphomas 'nasal type': an Italian multicentric retrospective survey Ann. Onc., May 1, 2006; 17(5): 794 - 800. [Abstract] [Full Text] [PDF]

				R. Willemze, E. S. Jaffe, G. Burg, L. Cerroni, E. Berti, S. H. Swerdlow, E. Ralfkiaer, S. Chimenti, J. L. Diaz-Perez, L. M. Duncan, et al. WHO-EORTC classification for cutaneous lymphomas Blood, May 15, 2005; 105(10): 3768 - 3785. [Abstract] [Full Text] [PDF]

				T.-t. Kuo, L.-Y. Shih, and N.-M. Tsang Nasal NK/T Cell Lymphoma in Taiwan: A Clinicopathologic Study of 22 Cases, with Analysis of Histologic Subtypes, Epstein-Barr Virus LMP-1 Gene Association, and Treatment Modalities International Journal of Surgical Pathology, October 1, 2004; 12(4): 375 - 387. [Abstract] [PDF]

				D. P. Beynet, S. A. Wee, S. S. Horwitz, S. Kohler, S. Horning, R. Hoppe, and Y. H. Kim Clinical and Pathological Features of Posttransplantation Lymphoproliferative Disorders Presenting With Skin Involvement in 4 Patients Arch Dermatol, September 1, 2004; 140(9): 1140 - 1146. [Abstract] [Full Text] [PDF]

				M. W. Bekkenk, P. M. Jansen, C. J. L. M. Meijer, and R. Willemze CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature Ann. Onc., July 1, 2004; 15(7): 1097 - 1108. [Abstract] [Full Text] [PDF]

				CD56+ Lymphoma With Skin Involvement: Clinicopathologic Features and Classification Arch Dermatol, April 1, 2004; 140(4): 427 - 436.

				J. P. Greer, M. C. Kinney, and T. P. Loughran Jr. T Cell and NK Cell Lymphoproliferative Disorders Hematology, January 1, 2001; 2001(1): 259 - 281. [Abstract] [Full Text] [PDF]