title: Results of treatment of advanced-stage lymphoblastic lymphoma at St Jude Children's Research Hospital from 1962 to 2002.
abstract: BACKGROUND
  Reliable prognostic factors have not been established for advanced-stage pediatric lymphoblastic lymphoma (LL). We analyzed treatment outcomes and potential risk factors in children and adolescents with advanced-stage LL treated over a 40-year period.
  
  
  METHODS
  From 1962 through 2002, 146 patients (99 boys and 47 girls) with stage III (n = 111) or stage IV (n = 35) LL were treated at St Jude Children's Research Hospital. The five treatment eras were 1962-1975 (no protocol), 1975-1979 (NHL-75), 1979-1984 (Total 10 High), 1985-1992 (Pediatric Oncology Group protocol), and 1992-2002 (NHL13). Age at diagnosis was <10 years in 65 patients and ≥10 years in 81.
  
  
  RESULTS
  Outcomes improved markedly over successive treatment eras. NHL13 produced the highest 5-year event-free survival (EFS) estimate (82.9% ± 6.1% [SE]) compared with only 20.0% ± 8.0% during the earliest era. Treatment era (P < 0.0001) and age at diagnosis (<10 years versus ≥10 years, P = 0.0153) were independent prognostic factors, whereas disease stage, lactate dehydrogenase level, and presence of a pleural effusion were not.
  
  
  CONCLUSIONS
  Treatment era and age were the most important prognostic factors for children with advanced-stage LL. We suggest that a better assessment of early treatment response may help to identify patients with drug-resistant disease who require more intensive therapy.
authors.affiliation: Department of Oncology, St Jude Children’s Research Hospital, TN 38105-3678, USA. john.sandlund@stjude.org
authors.forename: J T
authors.initials: JT
authors.lastname: Sandlund
authors.forename: C H
authors.initials: CH
authors.lastname: Pui
authors.forename: Y
authors.initials: Y
authors.lastname: Zhou
authors.forename: M
authors.initials: M
authors.lastname: Onciu
authors.forename: D
authors.initials: D
authors.lastname: Campana
authors.forename: M M
authors.initials: MM
authors.lastname: Hudson
authors.forename: H
authors.initials: H
authors.lastname: Inaba
authors.forename: M L
authors.initials: ML
authors.lastname: Metzger
authors.forename: D
authors.initials: D
authors.lastname: Bhojwani
authors.forename: R C
authors.initials: RC
authors.lastname: Ribeiro
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D018572:Disease-Free Survival; D019008:Drug Resistance, Neoplasm; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000401:mortality*; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: adolescent; advanced stage; children; lymphoblastic lymphoma; prognostic factor; treatment