title: Extranodal natural killer/T-cell lymphoma from skin or soft tissue: suggestion of treatment from multinational retrospective analysis.
abstract: BACKGROUND
  Clinical features and outcomes of extranodal natural killer/T-cell lymphoma (ENKL) arising from extranasal sites are not fully understood. The purpose of this study was to study the prognosis and treatment outcome of skin/soft tissue primary ENKL.
  
  
  METHODS
  This multicenter retrospective study included 48 patients with skin/soft tissue primary ENKL diagnosed from 1993 to 2010.
  
  
  RESULTS
  Patients with Ann Arbor stage I, T1-2N0M0 by International Society for Cutaneous Lymphomas-European Organization of Research and Treatment of Cancer TNM (tumour-node-metastasis) stage, International prognostic index score of 0-1, and a Korean prognostic index (KPI) score of 0-1 were associated with better survival. Four of five patients with T1-2N0M0 disease achieved complete response with radiation alone. In disseminated disease, only 6 of 13 patients responded to anthracycline-containing chemotherapy, and all the two patients receiving SMILE showed response.
  
  
  CONCLUSIONS
  In conclusion, we identified the prognostic value of KPI, and we suggest a treatment recommendation according to the TNM (tumour-node-metastasis) stage. Radiotherapy with/without chemotherapy seemed to be optimal in localized disease. In advanced stages, a more aggressive treatment regimen with newer agents should be sought.
authors.affiliation: Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul.
authors.forename: H K
authors.initials: HK
authors.lastname: Ahn
authors.affiliation: Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: C
authors.initials: C
authors.lastname: Suh
authors.affiliation: Department of Pathology, Chi-Mei Medical Center, Tainan and Taipei Medical University, Taipei, Taiwan.
authors.forename: S S
authors.initials: SS
authors.lastname: Chuang
authors.affiliation: Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: J
authors.initials: J
authors.lastname: Suzumiya
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul.
authors.forename: Y H
authors.initials: YH
authors.lastname: Ko
authors.affiliation: Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul.
authors.forename: S J
authors.initials: SJ
authors.lastname: Kim
authors.affiliation: Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul.
authors.forename: J R
authors.initials: JR
authors.lastname: Huh
authors.affiliation: Department of Pathology, Chi-Mei Medical Center, Tainan and Taipei Medical University, Taipei, Taiwan.
authors.forename: D H
authors.initials: DH
authors.lastname: Yoon
authors.affiliation: Department of Internal Medicine, Dong-A University College of Medicine, Busan.
authors.forename: S Y
authors.initials: SY
authors.lastname: Oh
authors.affiliation: Division of Hematology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul.
authors.forename: J S
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Dankook University College of Medicine, Cheonan, Korea.
authors.forename: S I
authors.initials: SI
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, Dankook University College of Medicine, Cheonan, Korea.
authors.forename: K W
authors.initials: KW
authors.lastname: Park
authors.affiliation: Department of Pathology and Laboratory Medicine, Kaohsiung Veterans General Hospital, and Center for General Education, Yuh-Ing Junior College of Health Care and Management, Kaohsiung, Taiwan.
authors.forename: P P
authors.initials: PP
authors.lastname: Hsieh
authors.affiliation: Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya.
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama.
authors.forename: T
authors.initials: T
authors.lastname: Yoshino
authors.affiliation: Department of Dermatology, Niigata University Graduate School of Medicine, Niigata.
authors.forename: K
authors.initials: K
authors.lastname: Ito
authors.affiliation: Department of Dermatology, Yokohama City University Medical Center, Yokohama.
authors.forename: T
authors.initials: T
authors.lastname: Nagatani
authors.affiliation: Department of Hematology, Juntendo University, Tokyo.
authors.forename: K
authors.initials: K
authors.lastname: Oshimi
authors.affiliation: Department of HSCT Data Management, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Suzuki
authors.affiliation: Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul. Electronic address: wskimsmc@skku.edu.
authors.forename: W S
authors.initials: WS
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology*; D016399:Lymphoma, T-Cell / Q000276:immunology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000276:immunology* / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000276:immunology* / Q000628:therapy; D055815:Young Adult