1. Primary cutaneous NK/T-cell lymphoma, nasal type and CD56-positive peripheral T-cell lymphoma: a cellular lineage and clinicopathologic study of 60 patients from Asia.
- Author
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Takata K, Hong ME, Sitthinamsuwan P, Loong F, Tan SY, Liau JY, Hsieh PP, Ng SB, Yang SF, Pongpruttipan T, Sukpanichnant S, Kwong YL, Hyeh Ko Y, Cho YT, Chng WJ, Matsushita T, Yoshino T, and Chuang SS
- Subjects
- Asia epidemiology, Biomarkers, Tumor genetics, Biopsy, DNA, Viral analysis, Diagnosis, Differential, Female, GPI-Linked Proteins analysis, Gene Rearrangement, Genes, T-Cell Receptor, Herpesvirus 4, Human genetics, Humans, Immunohistochemistry, In Situ Hybridization, Kaplan-Meier Estimate, Lymphoma, Extranodal NK-T-Cell ethnology, Lymphoma, Extranodal NK-T-Cell genetics, Lymphoma, Extranodal NK-T-Cell mortality, Lymphoma, Extranodal NK-T-Cell pathology, Lymphoma, Extranodal NK-T-Cell virology, Lymphoma, T-Cell, Peripheral ethnology, Lymphoma, T-Cell, Peripheral genetics, Lymphoma, T-Cell, Peripheral mortality, Lymphoma, T-Cell, Peripheral pathology, Lymphoma, T-Cell, Peripheral virology, Male, Middle Aged, Multivariate Analysis, Neoplasm Staging, Predictive Value of Tests, Receptors, IgG analysis, Retrospective Studies, Risk Factors, Skin Neoplasms ethnology, Skin Neoplasms genetics, Skin Neoplasms mortality, Skin Neoplasms pathology, Skin Neoplasms virology, Asian People genetics, Biomarkers, Tumor analysis, CD56 Antigen analysis, Cell Lineage, Lymphoma, Extranodal NK-T-Cell immunology, Lymphoma, T-Cell, Peripheral immunology, Skin Neoplasms immunology
- Abstract
Primary cutaneous, extranodal natural killer/T-cell lymphoma, nasal type (PC-ENKTL), is a rare Epstein-Barr virus (EBV)-associated neoplasm with poorly defined clinicopathologic features. We performed a multinational retrospective study of PC-ENKTL and CD56-positive EBV-negative peripheral T-cell lymphoma (PC-CD56+PTCL) in Asia in an attempt to elucidate their clinicopathologic features. Using immunohistochemistry for T-cell receptors (TCRs), in situ hybridization for EBV, and TCR gene rearrangement, we classified 60 tumors into 51 with PC-ENKTL (20 of NK-cell, 17 T-cell, and 14 indeterminate lineages) and 9 with PC-CD56+PTCL. Tumors of T-cell origin accounted for 46% of PC-ENKTLs with half of these cases being TCR-silent. As compared with T-lineage tumors, PC-ENKTLs of NK-cell lineage had more frequent involvement of regional lymph nodes and more frequently CD8-negative and CD56-positive. Cases of PC-ENKTL showed more frequent tumor necrosis, younger age, and a higher frequency of CD16 and CD30 expression than cases of PC-CD56+PTCL. CD56-positive T-lineage PC-ENKTL tumors (n=8) had more localized disease in the TNM (tumor-node-metastasis) staging and were more often of γδ T-cell origin compared with cases of PC-CD56+PTCL (n=9). PC-ENKTLs and PC-CD56+PTCLs were equally aggressive, with a 5-year overall survival rate of 25%. Tumor necrosis and CD16 expression may serve as useful surrogates for differentiating PC-ENKTL from PC-CD56+PTCL. A single lesion, an elevated lactate dehydrogenase level, and the presence of B symptoms were independent poor prognostic factors for PC-ENKTL in multivariate analysis. Further studies with more cases are warranted to delineate the clinicopathologic features and significance of EBV in these rare lymphomas.
- Published
- 2015
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