title: Clinicopathologic and molecular features in cutaneous extranodal natural killer-/T-cell lymphoma, nasal type, with aggressive and indolent course.
abstract: BACKGROUND
  Extranodal natural killer-/T-cell lymphoma, nasal type (ENKTCL-NT) is a highly aggressive lymphoma and prognosis is usually poor. The genetic background of primary cutaneous cases is poorly understood.
  
  
  OBJECTIVE
  We sought to evaluate the clinicopathologic features of cutaneous ENKTCL-NT, and the prognostic significance of genomic copy number alterations.
  
  
  METHODS
  Eight cases of cutaneous ENKTCL-NT (5 primary, 2 secondary, 1 no staging performed), including 2 patients with an unusually prolonged course of 5 and 23 years, were investigated using array comparative genomic hybridization.
  
  
  RESULTS
  All patients presented with typical clinicopathologic features. Epstein-Barr virus was found in neoplastic cells in all specimens. Copy number alterations were detected in all 8 cases with losses on 6q (37.5% of cases) and 7p (37.5% of cases), and gains on 7q (37.5% of cases) being the most frequent. Complexity of array comparative genomic hybridization profile did not correlate with the course of the disease. However, an increase of copy number alterations was detected in sequential biopsy specimens of 1 long-term survivor.
  
  
  CONCLUSIONS
  This was a small case series retrospective study.
  
  
  CONCLUSIONS
  Clinicopathologic features of cutaneous ENKTCL-NT are distinctive. Lower number of copy number alterations cannot be used as predictor for prolonged survival in cutaneous ENKTCL-NT.
authors.affiliation: Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.
authors.forename: Isabella
authors.initials: I
authors.lastname: Fried
authors.affiliation: Institute of Human Genetics, Medical University of Graz, Graz, Austria.
authors.forename: Monika
authors.initials: M
authors.lastname: Artl
authors.affiliation: Dermatopathology Unit, San Gallicano Dermatological Institute, Rome, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Cota
authors.affiliation: Department of Dermatology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Hansgeorg
authors.initials: H
authors.lastname: Müller
authors.affiliation: Dermatology Department, Hospital Garcia de Orta, Almada, Portugal.
authors.forename: Elvira
authors.initials: E
authors.lastname: Bartolo
authors.affiliation: Department of Pathology, Santa Chiara Hospital, Trento, Italy.
authors.forename: Sebastiana
authors.initials: S
authors.lastname: Boi
authors.affiliation: Department of Pathology, San Martino Hospital, Belluno, Italy.
authors.forename: Concetta
authors.initials: C
authors.lastname: Chiarelli
authors.affiliation: Departments of Dermatology and Pathology, Hospital da Luz, Lisbon, Portugal.
authors.forename: Esmeralda
authors.initials: E
authors.lastname: Vale
authors.affiliation: Department of Dermatology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Matthias
authors.initials: M
authors.lastname: Schmuth
authors.affiliation: Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.
authors.forename: Thomas
authors.initials: T
authors.lastname: Wiesner
authors.affiliation: Institute of Human Genetics, Medical University of Graz, Graz, Austria.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Speicher
authors.affiliation: Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria. Electronic address: lorenzo.cerroni@medunigraz.at.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
mesh_terms: D000293:Adolescent; D000328:Adult; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D020022:Genetic Predisposition to Disease / Q000453:epidemiology; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology*; D039102:Poly(A)-Binding Proteins / Q000235:genetics*; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D016019:Survival Analysis; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D039102:Poly(A)-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human
keywords: array comparative genomic hybridization; clinicopathologic features; cutaneous T-cell lymphoma; extranodal natural killer–/T-cell lymphoma; genomic aberrations; nasal type; prognosis