title: Extranodal natural killer/T-cell lymphoma, nasal type in Taiwan: a relatively higher frequency of T-cell lineage and poor survival for extranasal tumors.
abstract: Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a predominantly extranodal lymphoma associated with Epstein-Barr virus occurring most frequently in the upper aerodigestive tract. There are limited reports on cellular origin and prognostic factors. We retrospectively investigated 73 cases with a median age of 54 years and a male-female ratio of 2.0:1. The upper aerodigestive tract (nasal group) was the most common site of involvement (51 cases; 70%). The other organs (n = 22; extranasal group) included the skin (12 cases; 16%) and gastrointestinal tract (5; 7%). Of the 70 cases with complete staging, 71% had stage I/II disease. All cases were positive for Epstein-Barr virus by in situ hybridization. Using immunohistochemistry and clonality assay for T-cell receptor gene rearrangement, these tumors were classified into NK (n = 39; 53%), T (n = 13; 18%), and indeterminate lineage (n = 21; 29%). The only clinicopathological difference among these 3 groups was rare CD5 expression in the NK-cell group. Nasal tumors were more frequently of NK-cell origin, and extranasal tumors were equally of either T- or NK-cell origin. The 5-year overall survival rate was 35.6%. The overall survival time was shorter in the extranasal group, although there was no statistical difference in age, sex, and histologic or immunophenotypic features between the 2 groups. Excluding the cases with indeterminate lineage, 75% of cases were of NK lineage; and 25%, T lineage. Extranasal tumors were more aggressive than their nasal counterparts. A prospective national study is warranted for a better understanding of the clinicopathological and genetic features of this uncommon tumor and the prognostic factors.
authors.affiliation: Department of Anatomic Pathology, Far Eastern Hospital, New Taipei City, Taiwan, 22060. Electronic address: superelud@gmail.com.
authors.forename: Jie-Yang
authors.initials: JY
authors.lastname: Jhuang
authors.affiliation: Department of Pathology, Chi-Mei Medical Center, Tainan, Taiwan, 71004; Department of Nursing, National Tainan Institute of Nursing, Tainan, 71004. Electronic address: hoshifat@gmail.com.
authors.forename: Sheng-Tsung
authors.initials: ST
authors.lastname: Chang
authors.affiliation: Department of Medical Research, Chi-Mei Medical Center, Tainan, Taiwan, 71004. Electronic address: also99@gmail.com.
authors.forename: Shih-Feng
authors.initials: SF
authors.lastname: Weng
authors.affiliation: Department of Pathology, Miaoli General Hospital, Miaoli, Taiwan, 36054. Electronic address: rstpan@hotmail.com.
authors.forename: Shien-Tung
authors.initials: ST
authors.lastname: Pan
authors.affiliation: Department of Pathology, St. Martin De Porres Hospital, Chiayi, Taiwan, 60069; School of Medicine, Fu-Jen Catholic University, New Taipei City, Taiwan, 60069. Electronic address: chu.peiyi@msa.hinet.net.
authors.forename: Pei-Yi
authors.initials: PY
authors.lastname: Chu
authors.affiliation: Department of Pathology and Laboratory Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, and National Defense Medical Center, Taipei, Taiwan, 81362. Electronic address: pinpenh@gmail.com.
authors.forename: Pin-Pen
authors.initials: PP
authors.lastname: Hsieh
authors.affiliation: Department of Hemato-oncology, National Taiwan University Hospital, Hsin-Chu branch, Hsin-Chu, Taiwan, 30059. Electronic address: mdwei@yahoo.com.tw.
authors.forename: Chih-Hsin
authors.initials: CH
authors.lastname: Wei
authors.affiliation: Department of Pathology, Yuan's General Hospital, Kaohsiung, Taiwan, 80249. Electronic address: scchou_pat@yuanhosp.com.tw.
authors.forename: Shih-Cheng
authors.initials: SC
authors.lastname: Chou
authors.affiliation: Department of Pathology, Tungs' Taichung MetroHarbor Hospital, Taichung, Taiwan, 43304. Electronic address: kchiew58@gmail.com.
authors.forename: Chiew-Loon
authors.initials: CL
authors.lastname: Koo
authors.affiliation: Department of Surgical Pathology, Changhua Christian Hospital, Changhua, Taiwan, 50006; School of Medicine, Chung Shan Medical University, Taichung, Taiwan, 50006; Department of Medical Technology, Jen-Teh Junior College of Medicine, Nursing and Management, Miaoli, Taiwan, 50006. Electronic address: 132540@cch.org.tw.
authors.forename: Chih-Jung
authors.initials: CJ
authors.lastname: Chen
authors.affiliation: Department of Pathology, Chung Shan Medical University Hospital and School of Medicine, Chung Shan Medical University, Taichung, Taiwan, 40201. Electronic address: colin-dong@yahoo.com.tw.
authors.forename: Jeng-Dong
authors.initials: JD
authors.lastname: Hsu
authors.affiliation: Department of Pathology, Chi-Mei Medical Center, Tainan, Taipei Medical University and National Taiwan University, Taipei, Taiwan, 71004. Electronic address: cmh5301@mail.chimei.org.tw.
authors.forename: Shih-Sung
authors.initials: SS
authors.lastname: Chuang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019070:Cell Lineage / Q000276:immunology*; D005260:Female; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000473:pathology*; D013624:Taiwan
keywords: Epstein-Barr virus; Extranodal natural killer/T-cell lymphoma, nasal type; NK-cell lymphoma; Natural killer/T-cell lymphoma; Peripheral T-cell lymphoma; TCR gene rearrangement; Taiwan