title: Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis.
abstract: Extranodal natural killer/T-cell lymphoma, nasal type (NK/TCL) is more prevalent in Asia and in some areas of South and Central America, but it is rarely seen in the United States and Europe. In this study, a series of 122 cases of NK/TCL from Brazil was analyzed with respect to clinicopathologic features. Clinical characteristics and geographic distribution were evaluated in 97 cases of nasal/nasopharyngeal region and 23 cases in extranasal sites including 6 nodal cases. Clinical staging and follow-up information was available in a subset of 21 patients. All cases harbored Epstein-Barr virus (EBV), 95% and 85% expressed cytoplasmic CD3 and CD56, respectively, and all cases were positive for at least 1 marker for cytotoxic granules. The global distribution of EBV subtypes showed predominance of strain subtype A, 89%, and subtype B, 11%. No dual infections were detected. TCR-γ TCR-gene rearrangement was observed in 7 cases; all of them extranodal. Three of TCR-γ(+) cases showed EBV subtype A. Two TCR-γ(+)/CD56(+) cases showed EBV subtype B. Geographic distribution of NK/TCL showed higher frequency in the southeast and northeast regions of Brazil. Striking differences among geographic regions were seen with the vast majority of EBV subtype B (86%) occurring in the south and southeast regions.
authors.affiliation: Consultoria em Patologia, Botucatu, SP, Brazil.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Gualco
authors.forename: Pollyanna
authors.initials: P
authors.lastname: Domeny-Duarte
authors.forename: Lucimara
authors.initials: L
authors.lastname: Chioato
authors.forename: Glen
authors.initials: G
authors.lastname: Barber
authors.forename: Yasodha
authors.initials: Y
authors.lastname: Natkunam
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bacchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001938:Brazil / Q000453:epidemiology; D016009:Chi-Square Distribution; D002648:Child; D004279:DNA, Viral / Q000302:isolation & purification; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D019675:Genes, T-Cell Receptor gamma; D004854:Herpesvirus 4, Human / Q000145:classification* / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D053208:Kaplan-Meier Estimate; D008198:Lymph Nodes / Q000473:pathology / Q000821:virology; D054391:Lymphoma, Extranodal NK-T-Cell / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000821:virology*; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000821:virology*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D016133:Polymerase Chain Reaction; D011379:Prognosis; D012111:Residence Characteristics; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D046888:Tissue Array Analysis; D055815:Young Adult
chemical_list: D004279:DNA, Viral