title: Relapses in hepatoblastoma patients: clinical characteristics and outcome--experience of the International Childhood Liver Tumour Strategy Group (SIOPEL).
abstract: OBJECTIVE
  To analyse the clinical characteristics and outcome of hepatoblastoma (HB) patients who relapsed after enrolment on SIOPEL studies 1-3.
  
  
  METHODS
  Analysis of clinical data of all 59 patients (pts) registered in SIOPEL 1-3 studies, who relapsed after achieving complete remission (CR).
  
  
  RESULTS
  The median time from the initial diagnosis to relapse was 12 months (4-115 m). The site of relapse was lung N=27, liver N=21, both liver and lung N=5 and other N=5 (missing data-MD: 1 patient). All but 9 pts had an alpha-fetoprotein level >10 ng/mL at the time of relapse. Treatment of the relapse included chemotherapy and surgery N=25, chemotherapy alone N=21, surgery alone N=7 and only palliative treatment N=5 (MD: 1 pt). Overall, 31 pts (52%) achieved a second CR. With a median follow-up of 83 months, 23 pts are alive, (18 in 2nd CR, 5 after a second relapse) and 36 pts have died (35 from disease and 1 from complications). Three-year event-free survival and overall survival are 34% and 43% respectively (95% confidence interval [CI] 0.28-0.69). The main factors associated with a good outcome were PRETEXT group I-III at diagnosis, a high AFP level at relapse and relapse treatment including both chemotherapy and surgery.
  
  
  CONCLUSIONS
  Relapses in HB are rare events occurring in less than 12% of pts after CR. Combined treatment with chemotherapy and surgical removal of the tumour is essential for long-term survival.
authors.affiliation: Department of Pediatrics, Gustave Roussy Institut, Villejuif, France.
authors.forename: M
authors.initials: M
authors.lastname: Semeraro
authors.forename: S
authors.initials: S
authors.lastname: Branchereau
authors.forename: R
authors.initials: R
authors.lastname: Maibach
authors.forename: J
authors.initials: J
authors.lastname: Zsiros
authors.forename: M
authors.initials: M
authors.lastname: Casanova
authors.forename: P
authors.initials: P
authors.lastname: Brock
authors.forename: C
authors.initials: C
authors.lastname: Domerg
authors.forename: D C
authors.initials: DC
authors.lastname: Aronson
authors.forename: A
authors.initials: A
authors.lastname: Zimmermann
authors.forename: V
authors.initials: V
authors.lastname: Laithier
authors.forename: M
authors.initials: M
authors.lastname: Childs
authors.forename: D
authors.initials: D
authors.lastname: Roebuck
authors.forename: G
authors.initials: G
authors.lastname: Perilongo
authors.forename: P
authors.initials: P
authors.lastname: Czauderna
authors.forename: L
authors.initials: L
authors.lastname: Brugieres
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D016879:Salvage Therapy; D015996:Survival Rate