title: Initial liver transplantation for unresectable hepatoblastoma after chemotherapy.
abstract: BACKGROUND
  SIOPEL protocols have recommended liver transplantation for unresectable hepatoblastoma (HBL) after chemotherapy in absence of visible extrahepatic disease.
  
  
  METHODS
  This retrospective single center study includes 13 children treated following SIOPEL 3 or 4 protocols who underwent orthotopic liver transplantation (OLT) for HBL between February 2001 and May 2009.
  
  
  RESULTS
  Twelve patients had PRETEXT IV HBL, one had PRETEXT II P + HBL, two had pulmonary metastasis at diagnosis. Extra hepatic vascular involvement was present in seven patients (two vena cava, four main portal vein). Twelve patients received a deceased donor organ graft; wait time to OLT was 16 days (1-50 days). One patient received a living donor graft. Four patients did not undergo post-OLT chemotherapy because of major post-OLT surgical complications. Mean follow up was 3.1 years (1-5 years). Ten patients are alive, eight in first complete remission (CR), one is in second CR after two surgical pulmonary metastasis were removed, the latter is in second CR after a surgery for excision of two local recurrences and re-OLT for a secondary HBL in the first graft. Three patients died (two from tumor recurrence, one from cardiac failure after second OLT). Overall survival at 1 and 4 years was 100% and 83.3%.
  
  
  CONCLUSIONS
  Application of SIOPEL protocols for treatment of HBL in a specialized multidisciplinary team with access to liver transplantation has resulted in excellent survival. Initial extrahepatic disease should not be considered a contraindication. Future refinements of the protocol need to be considered to reduce toxicity.
authors: Paediatric Surgery APHP-Bicêtre Hospital University of Paris Sud XI, France.
authors: Géraldine
authors: G
authors: Héry
authors: Stéphanie
authors: S
authors: Franchi-Abella
authors: Dalila
authors: D
authors: Habes
authors: Laurence
authors: L
authors: Brugières
authors: Hélène
authors: H
authors: Martelli
authors: Monique
authors: M
authors: Fabre
authors: Danièle
authors: D
authors: Pariente
authors: Frédéric
authors: F
authors: Gauthier
authors: Emmanuel
authors: E
authors: Jacquemin
authors: Sophie
authors: S
authors: Branchereau
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018197:Hepatoblastoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome