title: 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.
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.
authors.affiliation: *Department of Pathology, Okayama University Graduate School of Medicine Medical School, Okayama ***Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan †Department of Pathology, Samsung Medical Center ∥∥Department of Pathology, Samsung Medical Center and Sungkyunkwan University, Seoul, Korea ‡Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Departments of §Pathology §§Medicine, Queen Mary Hospital, Hong Kong SAR, China ∥Department of Pathology, Singapore General Hospital Departments of **Pathology ##Haematology-Oncology, National University Health System ††Cancer Science Institute of Singapore, National University of Singapore, Singapore ¶Department of Pathology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan †††Department of Pathology, Chi-Mei Medical Center, Tainan, Taipei Medical University and National Taiwan University ¶¶Department of Dermatology, National Taiwan University Hospital, Taipei #Department of Pathology and Laboratory Medicine, Kaohsiung Veterans General Hospital ‡‡Department of Pathology, Kaohsiung Medical University Hospital and College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Min-Eui
authors.initials: ME
authors.lastname: Hong
authors.forename: Panitta
authors.initials: P
authors.lastname: Sitthinamsuwan
authors.forename: Florence
authors.initials: F
authors.lastname: Loong
authors.forename: Soo-Yong
authors.initials: SY
authors.lastname: Tan
authors.forename: Jau-Yu
authors.initials: JY
authors.lastname: Liau
authors.forename: Pin-Pen
authors.initials: PP
authors.lastname: Hsieh
authors.forename: Siok-Bian
authors.initials: SB
authors.lastname: Ng
authors.forename: Sheau-Fang
authors.initials: SF
authors.lastname: Yang
authors.forename: Tawatchai
authors.initials: T
authors.lastname: Pongpruttipan
authors.forename: Sanya
authors.initials: S
authors.lastname: Sukpanichnant
authors.forename: Yok-Lam
authors.initials: YL
authors.lastname: Kwong
authors.forename: Young
authors.initials: Y
authors.lastname: Hyeh Ko
authors.forename: Yung-Tsu
authors.initials: YT
authors.lastname: Cho
authors.forename: Wee Joo
authors.initials: WJ
authors.lastname: Chng
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
authors.forename: Shih-Sung
authors.initials: SS
authors.lastname: Chuang
mesh_terms: D001208:Asia / Q000453:epidemiology; D044466:Asian People / Q000235:genetics; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D001706:Biopsy; D019002:CD56 Antigen / Q000032:analysis*; D019070:Cell Lineage; D004279:DNA, Viral / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D058851:GPI-Linked Proteins / Q000032:analysis; D015321:Gene Rearrangement; D019672:Genes, T-Cell Receptor; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D053208:Kaplan-Meier Estimate; D054391:Lymphoma, Extranodal NK-T-Cell / Q000208:ethnology / Q000235:genetics / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000821:virology; D016411:Lymphoma, T-Cell, Peripheral / Q000208:ethnology / Q000235:genetics / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D011237:Predictive Value of Tests; D017452:Receptors, IgG / Q000032:analysis; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000208:ethnology / Q000235:genetics / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000821:virology
chemical_list: D014408:Biomarkers, Tumor; D019002:CD56 Antigen; D004279:DNA, Viral; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; C522918:NCAM1 protein, human; D017452:Receptors, IgG