title: Survival after relapse in children with solid tumors: a follow-up study from the Italian off-therapy registry.
abstract: BACKGROUND
  Despite the increased survival of children with solid tumors, a significant proportion of cases still relapse following treatment discontinuation, and knowledge about the long-term outcome of this selected group of patients remains incomplete.
  
  
  OBJECTIVE
  To describe the long-term outcome of children treated for a solid tumor who relapsed after the elective end of therapy, and to explore factors associated with survival.
  
  
  METHODS
  All patients with the selected diagnoses-Hodgkin disease (HD), neuroblastoma (NB), tumor of the central nervous system (CNS), Wilms tumor (WT), or soft tissue sarcoma (STS)-enrolled in the Italian Pediatric Off-Therapy Registry in the period 1980-1998 were evaluated. Out of 3,927 patients, 694 had relapsed after treatment suspension; 639 were available for analysis. Survival and event-free survival were estimated by the Kaplan-Meier method. The log-rank test was used to assess differences in survival among the various types of cancer considered. Multivariate Cox proportional hazards analysis was adopted to explore possible prognostic factors.
  
  
  RESULTS
  There were 335 deaths: most of them (93%) were related to the primary cancer. The overall survival rate after relapse was 38% (95% CI 33-42) at 5 years, and 32% (95% CI 27-36%) at 15 years, while event free survival was 31% (95% CI 26-35) and 26% (95% CI 22-30%), respectively. There were significant differences according to the original diagnosis, with patients with HD doing better, and those with NB, CNS, and STS worse. No improvement of prognosis was evident over time. Post-relapse stem cell transplantation was associated with decreased risk of death only in the first year, not thereafter.
  
  
  CONCLUSIONS
  Overall, patients with solid tumors who relapse after treatment discontinuation have a poor outcome, but significant differences exist according to the tumor types.
authors.affiliation: Department of Pediatrics, S. Donà di Piave, Venezia, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ceschel
authors.forename: Veronica
authors.initials: V
authors.lastname: Casotto
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Valsecchi
authors.forename: Paolo
authors.initials: P
authors.lastname: Tamaro
authors.forename: Momcilo
authors.initials: M
authors.lastname: Jankovic
authors.forename: Guia
authors.initials: G
authors.lastname: Hanau
authors.forename: Franca
authors.initials: F
authors.lastname: Fossati
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
authors.forename: Roberto
authors.initials: R
authors.lastname: Rondelli
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sandri
authors.forename: Daniela
authors.initials: D
authors.lastname: Silvestri
authors.forename: Riccardo
authors.initials: R
authors.lastname: Haupt
authors.forename: Marina
authors.initials: M
authors.lastname: Cuttini
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007558:Italy; D008297:Male; D009423:Nervous System Neoplasms / Q000175:diagnosis* / Q000628:therapy; D009447:Neuroblastoma / Q000175:diagnosis* / Q000628:therapy; D011379:Prognosis; D012008:Recurrence; D012042:Registries; D012307:Risk Factors; D012509:Sarcoma / Q000175:diagnosis* / Q000628:therapy; D033581:Stem Cell Transplantation; D015996:Survival Rate; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000175:diagnosis* / Q000628:therapy