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NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status

 Y H Ko  ;  E-Y Cho  ;  J-E Kim  ;  S-S Lee  ;  J-R Huh  ;  H-K Chang  ;  W-I Yang  ;  C-W Kim  ;  S-W Kim  ;  H J Ree 
 HISTOPATHOLOGY, Vol.44(5) : 480-489, 2004-05 
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Issue Date
Adolescent ; Adult ; Aged ; Aged, 80 and over ; CD56 Antigen / metabolism ; Female ; Gastrointestinal Neoplasms / genetics ; Gastrointestinal Neoplasms / pathology ; Gastrointestinal Neoplasms / virology ; Gene Rearrangement ; Head and Neck Neoplasms / genetics ; Head and Neck Neoplasms / pathology ; Head and Neck Neoplasms / virology ; Herpesvirus 4, Human / isolation & purification* ; Humans ; In Situ Hybridization ; Killer Cells, Natural* ; Lymphoma, T-Cell, Peripheral / diagnosis ; Lymphoma, T-Cell, Peripheral / genetics ; Lymphoma, T-Cell, Peripheral / metabolism ; Lymphoma, T-Cell, Peripheral / mortality ; Lymphoma, T-Cell, Peripheral / pathology* ; Lymphoma, T-Cell, Peripheral / virology ; Male ; Middle Aged ; Necrosis / pathology ; Parotid Neoplasms / genetics ; Parotid Neoplasms / pathology ; Parotid Neoplasms / virology ; Poly(A)-Binding Proteins ; Prognosis ; Proteins / metabolism ; RNA, Viral / analysis ; RNA-Binding Proteins ; Soft Tissue Neoplasms / genetics ; Soft Tissue Neoplasms / pathology ; Soft Tissue Neoplasms / virology ; Survival Analysis ; T-Cell Intracellular Antigen-1 ; Testicular Neoplasms / genetics ; Testicular Neoplasms / pathology ; Testicular Neoplasms / virology
Aims: To analyse the clinicopathological findings of extranasal CD56+ cytotoxic T- or NK-cell lymphomas in different organs and to compare Epstein-Barr virus (EBV)+ and EBV- lymphoma of non-blastoid cytomorphology. Methods and results: Fifty-one cases of cCD3+ T-cell intracellular antigen (TIA-1)+ CD56+ lymphomas of extranodal/extranasal origin were included in the study. The primary sites of the CD56+ tumours were soft tissue (n = 10), the gastrointestinal (GI) tract (n = 13), the skin (n = 15), upper aerodigestive tract excluding nasal and nasopharyngeal regions (n = 11), the testis (n = 1), and parotid gland (n = 1). TCR gene rearrangement was detected in seven of 47 cases examined (16%). EBV was positive in 39 of 51 cases (76%). The positive rate of EBV was higher in tumours of soft tissue (80%), GI tract (92%), and skin (80%), and lowest in the upper aerodigestive tract excluding the nasal and nasopharyngeal region (50%). Tumours of the soft tissue and the upper aerodigestive tract tended to present with localized disease (P = 0.002). The 2-year survival rate was lowest for tumours of the GI tract (P = 0.0256). EBV- TCR- lymphoma showed less necrosis (P = 0.0133) and a better 2-year survival rate (P = 0.0066) than EBV+ TCR- lymphoma. Patients with EBV+ TCR+ lymphomas tended to present with localized disease, more often than EBV+ TCR- lymphoma (P = 0.0186). Significant prognostic factors in all CD56+ lymphomas were the site (P = 0.0256), EBV status (P = 0.0026), necrosis with or without perforation (P = 0.0338) and the presence of pleomorphic large tumour cells (P = 0.0428). Cox's regression analysis adjusting for other pathological parameters showed EBV status to be the only independent prognostic factor (P = 0.018). Conclusions: Extranodal CD56+ EBV- lymphoma at extranasal sites is a clinically less aggressive malignancy and displays less necrosis than CD56+ EBV+ lymphoma. Because CD56+ EBV+ TCR+ lymphomas show similar pathological and clinical findings to CD56+ EBV+ TCR- lymphomas, nasal-type NK/T-cell lymphomas at extranasal sites should be diagnosed as such on the basis of EBV+, cytotoxic T or NK phenotype irrespective of the genotype determined by molecular study.
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1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
Yang, Woo Ick(양우익) ORCID logo https://orcid.org/0000-0002-6084-5019
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