title: Orthotopic liver transplantation for unresectable hepatoblastoma: a single center's experience.
abstract: OBJECTIVE
  Complete surgical resection after chemotherapy is the definitive treatment for hepatoblastoma. However, orthotopic liver transplantation (OLT) is now accepted as a treatment modality for patients with unresectable tumours. The aim of this study was to review a single center's experience of OLT for unresectable hepatoblastoma.
  
  
  METHODS
  A retrospective review of 8 patients with unresectable hepatoblastoma who were referred for liver transplantation was conducted.
  
  
  RESULTS
  The patients assessed had an age range of 5 to 105 months at presentation; median, 24 months, (5 boys; 3 girls). Two patients have familial adenomatous polyposis, and one has right hemihypertrophy. All 8 patients had received standard chemotherapy according to SIOP (International Society of Pediatric Oncology) protocols. Extrahepatic metastases were found in 3 patients at diagnosis, but none had detectable metastases at the time of OLT. Four patients continued chemotherapy while awaiting OLT. Three patients received whole grafts, and 5 received reduced grafts. The median follow-up period was 22 months (range, 2 to 78 months). Five patients are alive and well, although 1 patient had a second OLT for biliary cirrhosis secondary to biliary stricture at 6 years. Three patients died: one 26 days post OLT of sepsis and two of disease recurrence at 22 months and 70 months posttransplant. The actuarial survival rate is 88% and 65% at 1 and 5 years, respectively, whereas the overall survival rate is 62.5%.
  
  
  CONCLUSIONS
  OLT for unresectable hepatoblastoma without extra hepatic metastases is highly successful with a low recurrence rate.
authors.affiliation: Department of Oncology, The Birmingham Children's Hospital NHS Trust, England.
authors.forename: W
authors.initials: W
authors.lastname: Al-Qabandi
authors.forename: H C
authors.initials: HC
authors.lastname: Jenkinson
authors.forename: J A
authors.initials: JA
authors.lastname: Buckels
authors.forename: A D
authors.initials: AD
authors.lastname: Mayer
authors.forename: P
authors.initials: P
authors.lastname: McKiernan
authors.forename: B
authors.initials: B
authors.lastname: Morland
authors.forename: P
authors.initials: P
authors.lastname: John
authors.forename: D
authors.initials: D
authors.lastname: Kelly
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018197:Hepatoblastoma / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate