title: [Extranodal nasal type NK/T-cell lymphoma: clinicopathologic and prognostic study of 55 cases].
abstract: OBJECTIVE
  To study the clinicopathologic features and prognostic factors of extranodal nasal type NK/T-cell lymphoma (EN-NK/TCL) in Chinese patients.
  
  
  METHODS
  Fifty-five cases of EN-NK/TCL diagnosed in Chinese patients during the period from 1998 to 2007 were studied by light microscopy, immunohistochemistry and in-situ hybridization. The follow-up information was analyzed.
  
  
  RESULTS
  The male-to-female ratio was 1.89:1. The median age of the patients was 38 years. The commonest sites of involvement included nasal cavity and adjoining tissue (85.5%). Histologically, EN-NK/TCL was composed of small to medium-sized lymphoid cells. Angiocentric and angiodestructive growth patterns, coagulative tumor necrosis and apoptotic bodies were frequently observed. Immunohistochemical study showed that CD20, the B-cell marker, was negative in all cases. The positivity rates for T-cell markers CD3epsilon, CD4, CD5 and CD8 were 100% (49/49), 7% (3/46), 8% (4/48) and 63% (29/46), respectively. Most cases were also positive for NK-cell marker CD56 (79% 42/53). All cases expressed cytotoxic granule-associated proteins TIA-1 and granzyme B. Only 17% (8/46) of the cases were positive for anti-apoptotic protein bcl-2. The proliferation index, as demonstrated by Ki-67 immunostain, varied: 30% (14/47) with a low Ki-67 expression level (< or = 29%), 28% (13/47) with a medium level (30%-59%) and 42% with a high level (> or = 60%). There was a significant positive correlation between the bcl-2 positive expression and a high Ki-67 expression level. In-situ hybridization for EBV-encoded RNA was positive in all cases. Amongst the 41 cases with clinical information available, 63.4% presented with Ann Arbor stage I to II. The performance status score was 1 in 87.8% cases. High lactate dehydrogenase level was demonstrated in some patients (31.8%). Amongst the 27 cases with follow-up data available, the median survival was 13 months. The overall 1-year, 2-year and 5-year survival rates were 52%, 31% and 20%, respectively. In general, cases with high proliferation index carried poor prognosis.
  
  
  CONCLUSIONS
  EN-NK/TCL is a mature T-cell and NK-cell neoplasm which can be accurately diagnosed by histologic examination, immunohistochemical study and in-situ hybridization. The prognosis is usually not favorable. Proliferation index of the tumor represents an independent prognostic factor.
authors: Department of Pathology and Laboratory Medicine, Guangdong General Hospital (Guangdong Academy of Medicine Science), Guangzhou 510080, China.
authors: Li
authors: L
authors: Li
authors: Yan-hui
authors: YH
authors: Liu
authors: Heng-guo
authors: HG
authors: Zhuang
authors: Xin-lan
authors: XL
authors: Luo
authors: Fen
authors: F
authors: Zhang
authors: Fang-ping
authors: FP
authors: Xu
authors: Dong-lan
authors: DL
authors: Luo
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017252:CD3 Complex / Q000378:metabolism*; D019002:CD56 Antigen / Q000378:metabolism; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000821:virology; D005260:Female; D005500:Follow-Up Studies; D053804:Granzymes / Q000378:metabolism; D004854:Herpesvirus 4, Human; D006801:Humans; D016130:Immunophenotyping; D019394:Ki-67 Antigen / Q000378:metabolism; D054391:Lymphoma, Extranodal NK-T-Cell / Q000378:metabolism / Q000473:pathology / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000378:metabolism / Q000473:pathology / Q000628:therapy / Q000821:virology; D039102:Poly(A)-Binding Proteins / Q000378:metabolism; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D012367:RNA, Viral / Q000032:analysis; D012189:Retrospective Studies; D015996:Survival Rate; D000074019:T-Cell Intracellular Antigen-1; D055815:Young Adult
chemical_list: D017252:CD3 Complex; C115335:CD3E protein, human; D019002:CD56 Antigen; D019394:Ki-67 Antigen; D039102:Poly(A)-Binding Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D012367:RNA, Viral; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D053804:Granzymes