title: Prognostic Impact of Intensified Maintenance Therapy on Children With Advanced Lymphoblastic Lymphoma: A Report From the Japanese Pediatric Leukemia/Lymphoma Study Group ALB-NHL03 Study.
abstract: BACKGROUND
  Childhood advanced lymphoblastic lymphoma (LBL) has a favorable outcome with an event-free survival (EFS) rate of over 80% in response to treatment strategies for acute lymphoblastic leukemia (ALL). However, no progress has been made in this outcome over the past 10 years.
  
  
  METHODS
  We conducted the first nationwide prospective study of childhood advanced LBL to assess the efficacy and safety of ALL-directed therapy with an intensified maintenance phase. We omitted local radiotherapy including prophylactic cranial radiotherapy except for patients with initial central nervous system disease. The total duration of the treatment was 24 months.
  
  
  RESULTS
  For the 136 patients analyzed in this study, 5-year overall survival (OS) was 82.9% and 5-year EFS was 77.9%. Thirty events were observed and 14 occurred before the initiation of intensified maintenance phase. Of 14 events, nine were observed as mediastinal enlargement. There was no significant difference in outcome when stratified according to gender or by immunophenotype. The 5-year EFS according to clinical stage in patients with T-cell LBL (T-LBL) was 70.6% for stage III and 88.9% for stage IV (P = 0.037).
  
  
  CONCLUSIONS
  Our first nationwide study provided about 80% cure rate with only one case of toxic death in childhood advanced LBL. However, our intensified maintenance therapy could not improve the survival outcome. There was a trend of better EFS in Japanese patients with T-LBL stage IV than T-LBL stage III.
authors.affiliation: Department of Pediatrics, Japanese Red Cross Narita Hospital, Narita, Japan.
authors.forename: Shosuke
authors.initials: S
authors.lastname: Sunami
authors.affiliation: Department of Pediatrics, National Hospital Organization, Nagoya Medical Center, Aichi, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Sekimizu
authors.affiliation: Clinical Research Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Takimoto
authors.affiliation: Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Mori
authors.affiliation: Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Mitsui
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Reiji
authors.initials: R
authors.lastname: Fukano
authors.affiliation: Laboratory of Clinical, Epidemiological and Health Services Research, Clinical Research Center, National Hospital Organization, Nagoya Medical Center, Aichi, Japan.
authors.forename: Akiko Moriya
authors.initials: AM
authors.lastname: Saito
authors.affiliation: Department of Nutritional Science, Faculty of Psychological and Physical Science, Aichi Gakuin University, Aichi, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
authors.affiliation: Clinical Research Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Junichiro
authors.initials: J
authors.lastname: Fujimoto
authors.affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.affiliation: Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Japan.
authors.forename: Ryoji
authors.initials: R
authors.lastname: Kobayashi
authors.affiliation: Clinical Research Center, National Hospital Organization, Nagoya Medical Center, Aichi, Japan.
authors.forename: Keizo
authors.initials: K
authors.lastname: Horibe
authors.affiliation: Advanced Medical Research Center, Aichi Medical University, Aichi, Japan.
authors.forename: Masahito
authors.initials: M
authors.lastname: Tsurusawa
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D060046:Maintenance Chemotherapy / Q000379:methods*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000401:mortality; D011379:Prognosis; D011446:Prospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome
keywords: JPLSG (Japanese Pediatric Leukemia/Lymphoma Study Group); childhood; non-Hodgkin lymphoma; prognosis; treatment