title: Successful liver transplant for unresectable hepatoblastoma.
abstract: BACKGROUND
  Treatment of children with stage III and IV hepatoblastoma has shown little improvement with 5-year survival rates of 64% and 25%, respectively (J Clin Oncol 2000;18:2665-75). A timely and organized treatment program including preoperative chemotherapy combined with living donor liver transplantation and postoperative chemotherapy has been used seeking improved long-term survival in stage III and IV cases.
  
  
  METHODS
  A retrospective review of 8 patients with stage III and IV hepatoblastoma unresectable by conventional resection were treated with complete hepatectomy and transplantation. Approval was obtained from our institutional review board.
  
  
  RESULTS
  Since August of 2001, we have treated 6 patients with stage III hepatoblastoma and 2 patients with initial stage IV hepatoblastoma. These patients (age, 23 months-9 years) had all received extensive chemotherapy or prior resections. After chemotherapy, none had gross tumor documented outside of the liver at time of transplantation. All underwent hepatectomy including vena cava resection, in selected cases, with living donor orthotopic liver transplantation. All patients had at least 2 cycles of postoperative chemotherapy. Of 8 patients, 6 are alive and well with normalized alpha-fetoprotein levels. There were 2 late deaths from recurrent disease. Length of follow-up ranged from 7 to 53 months.
  
  
  CONCLUSIONS
  Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. This series indicates that children tolerate complete hepatectomy, transplantation, and postoperative chemotherapy well. Referral to a transplant center during the first 3 cycles of chemotherapy appears to offers the best opportunity for long-term survival.
authors.affiliation: Division of Solid Organ Transplant, AI duPont Hospital for Children, PO Box 269, Wilmington, DE 19899, USA. acasas@nemours.org
authors.forename: Adela T
authors.initials: AT
authors.lastname: Casas-Melley
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Malatack
authors.forename: Deborah
authors.initials: D
authors.lastname: Consolini
authors.forename: Keith
authors.initials: K
authors.lastname: Mann
authors.forename: Christopher
authors.initials: C
authors.lastname: Raab
authors.forename: Louise
authors.initials: L
authors.lastname: Flynn
authors.forename: Pamela
authors.initials: P
authors.lastname: Woolfrey
authors.forename: Jerome
authors.initials: J
authors.lastname: Menendez
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Dunn
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D009367:Neoplasm Staging; D012189:Retrospective Studies
chemical_list: D000970:Antineoplastic Agents