title: Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma--results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience.
abstract: OBJECTIVE
  Fibrolamellar hepatocellular carcinoma (FL-HCC) and conventional hepatocellular carcinoma (HCC) cases in two consecutive paediatric HCC trials were analysed to compare outcome and derive treatment implications.
  
  
  METHODS
  Data of 24 FL-HCC (24% PRETEXT IV) and 38 HCC (42% PRETEXT IV) cases from SIOPEL-2 and -3 (1995-1998, 1998-2006) were analysed. Patients were treated according to SIOPEL-2 and -3 high-risk protocol (carboplatin+doxorubicin alternating with cisplatin; seven preoperative, three postoperative cycles) or with primary surgery followed by chemotherapy as indicated.
  
  
  RESULTS
  Thirteen of 24 FL-HCC (54%) and 32/38 HCC (84%) were initially treated with chemotherapy. Eight FL-HCC (33%) and five HCC patients (13%) had primary surgery. Partial response was observed in 31% of FL-HCC versus 53% of HCC patients (p=0.17). Complete resection was achieved in ten FL-HCC and seven HCC patients (p=0.08). Three-year event free survival (EFS) was 22% for FL-HCC versus 28% for HCC. Overall survival (OS) was not significantly different at 3 years follow up (42% for FL-HCC versus 33% for HCC, p=0.24). EFS/OS Kaplan-Meier curves did not differ significantly, with median follow up of 43 (FL-HCC) and 60 (HCC) months. No significant correlation was found between potential prognostic factors and OS. In the entire cohort nine out of 23 (39%) patients with complete resection or orthotopic liver transplantation versus 34/39 (87%) without successful surgical treatment, died.
  
  
  CONCLUSIONS
  Long-term OS in FL-HCC and HCC is similar. With low response rates, complete resection remains the treatment of choice.
authors.affiliation: Department of Paediatric Surgery, Emma Children's Hospital, AMC, Amsterdam, The Netherlands. v.b.weeda@gmail.com
authors.forename: V B
authors.initials: VB
authors.lastname: Weeda
authors.forename: M
authors.initials: M
authors.lastname: Murawski
authors.forename: A J
authors.initials: AJ
authors.lastname: McCabe
authors.forename: R
authors.initials: R
authors.lastname: Maibach
authors.forename: L
authors.initials: L
authors.lastname: Brugières
authors.forename: D
authors.initials: D
authors.lastname: Roebuck
authors.forename: M
authors.initials: M
authors.lastname: Fabre
authors.forename: A
authors.initials: A
authors.lastname: Zimmermann
authors.forename: J B
authors.initials: JB
authors.lastname: Otte
authors.forename: M
authors.initials: M
authors.lastname: Sullivan
authors.forename: G
authors.initials: G
authors.lastname: Perilongo
authors.forename: M
authors.initials: M
authors.lastname: Childs
authors.forename: P
authors.initials: P
authors.lastname: Brock
authors.forename: J
authors.initials: J
authors.lastname: Zsíros
authors.forename: J
authors.initials: J
authors.lastname: Plaschkes
authors.forename: P
authors.initials: P
authors.lastname: Czauderna
authors.forename: D C
authors.initials: DC
authors.lastname: Aronson
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage; D006528:Carcinoma, Hepatocellular / Q000188:drug therapy* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D015331:Cohort Studies; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000187:drug effects* / Q000473:pathology / Q000601:surgery; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000601:surgery; D008297:Male; D016896:Treatment Outcome
chemical_list: D004317:Doxorubicin; D016190:Carboplatin; D002945:Cisplatin
keywords: Antineoplastic agents; Carcinoma; Clinical oncology; Fibrolamellar hepatocellular carcinoma; Hepatocellular; Hepatoma; Liver diseases; Liver neoplasms; Neoplasms; Paediatrics