title: Liver transplantation for malignant tumours in children.
abstract: OBJECTIVE
  The object of this study was to analyse our results with liver transplantation (LTX) for primitive malignant unresectable liver tumours in children and to discuss the controversial indications, based on our experience.
  
  
  METHODS
  We report on 12 patients, aged 6 months to 14 years, with hepatic malignant tumours: 11 with hepatoblastoma and 1 with fibrolamellar hepatocelullar carcinoma without cirrhosis. LTX was the primary treatment in 10 patients (PRETEXT IV or any grade, if there was extension to the retrohepatic vena cava, 3 hepatic veins or portal vein) and a rescue therapy after recurrence for 1 and for persistence of unresectable macroscopic residuals in 2 patients. One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (n = 5), left lateral segment from cadaveric donor (n = 3), living related donor (n = 3; 2 segments II-III and 1 right lobe) and left lateral segment from split liver (n = 1). All children received chemotherapy prior and post transplantation following the SIOPEL protocol. We analysed procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution.
  
  
  RESULTS
  All patients overcame LTX and no early graft loss was recorded. Two cases died because of tumour relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups, 90% and 50%). Graft and patient survival rates at 1 year, 3 years, 5 years and 14 years were 91%, 91%, 82% and 82% respectively. The boy who presented with lung metastases developed new ones one year after LTX that were removed and he is currently free of disease. The disease-free period has a probability at 1, 3 and 5 years of 91%, 75% and 75%, respectively. Tumour tissue persistence was the only risk factor for an adverse clinical course in our series.
  
  
  CONCLUSIONS
  LTX is a reasonable therapeutic approach for unresectable malignant liver tumours, providing outcomes comparable to those for resectable tumours. Results obtained with LTX are better when it is used as a primary treatment than when used as a rescue procedure. Proper staging and early referral to centres with enough expertise optimise the results. LTX for patients with lung metastases could be a controversial option. Living related-donor transplantation is an excellent alternative to avoid disease progression while on the waiting list for cadaveric donors.
authors.affiliation: Department of Paediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: L F
authors.initials: LF
authors.lastname: Avila
authors.forename: A L
authors.initials: AL
authors.lastname: Luis
authors.forename: F
authors.initials: F
authors.lastname: Hernandez
authors.forename: P
authors.initials: P
authors.lastname: Garcia Miguel
authors.forename: P
authors.initials: P
authors.lastname: Jara
authors.forename: A M
authors.initials: AM
authors.lastname: Andres
authors.forename: M
authors.initials: M
authors.lastname: Lopez Santamaría
authors.forename: J A
authors.initials: JA
authors.lastname: Tovar
mesh_terms: D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D019520:Living Donors; D008297:Male; D015996:Survival Rate