title: Survival after liver transplantation for hepatoblastoma: a 2-center experience.
abstract: OBJECTIVE
  Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy.
  
  
  METHODS
  All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, alpha-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels.
  
  
  RESULTS
  Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 +/- 48 months). Patients were transplanted a mean of 4 +/- 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival.
  
  
  CONCLUSIONS
  Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.
authors.affiliation: Department of Surgery, Children's Memorial Hospital, Feinberg School of Medicine, Northwestern University, Chicago, IL 60614, USA.
authors.forename: Marybeth
authors.initials: M
authors.lastname: Browne
authors.forename: Dani
authors.initials: D
authors.lastname: Sher
authors.forename: David
authors.initials: D
authors.lastname: Grant
authors.forename: Enza
authors.initials: E
authors.lastname: Deluca
authors.forename: Estella
authors.initials: E
authors.lastname: Alonso
authors.forename: Peter F
authors.initials: PF
authors.lastname: Whitington
authors.forename: Riccardo A
authors.initials: RA
authors.lastname: Superina
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000097:blood; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D000075202:Contraindications; D005260:Female; D005500:Follow-Up Studies; D018197:Hepatoblastoma / Q000097:blood / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000601:surgery*; D006776:Hospitals, Pediatric / Q000706:statistics & numerical data; D006785:Hospitals, University / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000097:blood / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality*; D008175:Lung Neoplasms / Q000401:mortality / Q000556:secondary; D008297:Male; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D018579:Patient Selection; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D000509:alpha-Fetoproteins / Q000032:analysis
chemical_list: C097353:AFP protein, human; D014408:Biomarkers, Tumor; D000509:alpha-Fetoproteins