Extranodal NK/T-Cell Lymphoma, Nasal-Type
Extranodal NK/T-cell lymphoma (eNK/TCL) is an aggressive Epstein-Barr virus (EBV)–associated lymphoma with a very poor prognosis. It commonly presents in the midline face or skin with large ulcerative nodules, may disseminate rapidly to the viscera and lymph nodes, and is complicated by hemophagocytic syndrome and disseminated intravascular coagulation (DIC). Alternative terms in the literature include angiocentric T-cell lymphoma, malignant midline reticulosis, lethal midline granuloma, polymorphic reticulosis, and angiocentric immunoproliferative lesion [1, 2]. One extremely important point to note is that this is not a lymphoma of natural killer T cells (NKT cells), but rather a neoplasm of NK cells or cells with a cytotoxic T-cell phenotype. Hence they are termed NK/T-cell lymphomas . Some differentiate eNK/TCLs that occur in the aerodigestive tract (nasal eNK/TCL) from those that occur elsewhere (nasal-type), but regardless of the primary site, the histology is identical . This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of eNK/TCL. The chapter closes with a clinical case featuring a patient with eNK/TCL arising in the nose with cutaneous metastases.
KeywordsExtranodal NK/T-cell lymphoma, nasal-type NK cell Cytotoxic T cell Nasal Opportunistic infection Hemophagocytic syndrome Disseminated intravascular coagulation Angiocentric Malignant midline reticulosis Midline granuloma Angioinvasive Angiodestructive Differential diagnosis Hydroa vacciniforme-like lymphoma Lymphomatoid granulomatosis CD8+ aggressive epidermotropic cutaneous T-cell lymphoma Blastic plasmacytoid dendritic cell neoplasm
- 1.Chan J, Quintanilla-Martinez L, Ferry J, Peh S-C. Extranodal NK/T-cell lymphoma, nasal type. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 285–8.Google Scholar
- 5.Elenitoba-Johnson KS, Zarate-Osorno A, Meneses A, Krenacs L, Kingma DW, Raffeld M, et al. Cytotoxic granular protein expression, Epstein-Barr virus strain type, and latent membrane protein-1 oncogene deletions in nasal T-lymphocyte/natural killer cell lymphomas from Mexico. Mod Pathol. 1998;11:754–61.PubMedGoogle Scholar
- 20.Kim SJ, Kim K, Kim BS, Kim CY, Suh C, Huh J, et al. Phase II trial of concurrent radiation and weekly cisplatin followed by VIPD chemotherapy in newly diagnosed, stage IE to IIE, nasal, extranodal NK/T-cell lymphoma: Consortium for Improving Survival of Lymphoma study. J Clin Oncol. 2009;27:6027–32.PubMedCrossRefGoogle Scholar
- 21.Yamaguchi M, Kwong Y-L, Kim WS, Maeda Y, Hashimoto C, Suh C, et al. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol. 2011;29:4410–6.PubMedCrossRefGoogle Scholar
- 27.Gaulard P, Berti E, Willemze R, Jaffe ES, et al. Primary cutaneous peripheral T-cell lymphomas, rare subtypes. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, editors. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer; 2008. p. 302–5.Google Scholar