title: Liver Transplantation for Malignant Primary Pediatric Hepatic Tumors.
abstract: BACKGROUND
  Malignant primary pediatric hepatic tumors (MPPHTs) are rare and account for approximately 1% of all childhood malignancies. In recent years, liver transplantation has emerged as a viable treatment options for select patients with MPPHTs.
  
  
  METHODS
  We performed a single-center retrospective study using a prospective database to compare outcomes of pediatric liver transplant recipients, with and without cancer, between January 2000 and December 2014.
  
  
  RESULTS
  One hundred fifty-three children underwent 173 liver transplantations during the study period. Of these, 21 (12%) children received 23 (13.3%) transplants for unresectable MPPHTs: 16 hepatoblastomas (HBs), 3 embryonal cell sarcomas (ECS), and 2 hepatocellular carcinomas (HCCs). There was no significant difference in 1-, 3-, and 10-year patient and graft survival rates between MPPHT and non-MPPHT patients (95.2%, 81.2%, 81.2%, and 95.2%, 72,2%, 72.2% for MPPHT vs 92.7%, 89.8%, 87.6% and 85.4%, 81.1%, 75% for the non-MPPHT group, respectively) (p > 0.05). Rates of 1-, 5-, and 10-year disease-free survival for MPPHT patients were 76%, 76%, and 76%, respectively. Median age at transplantation for MPPHT patients was 3.1 years (range 58 days to 17 years), median listing time was 81 days, and median wait list time was 15 days. Eight (38%) children had 2 tumors or more and 4 of 16 (25%) HB patients had metastatic disease at presentation. All children received neoadjuvant treatment, with radiographic response in 19 of 21 patients. Presence of metastatic HB at presentation, International Society of Pediatric Oncology Epithelial Liver (SIOPEL) high risk status, and persistently elevated alpha fetoprotein levels after neoadjuvant chemotherapy might be risk factors for tumor recurrence and decreased survival.
  
  
  CONCLUSIONS
  Liver transplantation is an excellent option for select patients with unresectable MPPHTs, with outcomes comparable to those after transplantation for nonmalignant causes.
authors.affiliation: Section of Transplant Surgery, Department of Surgery, Washington University, St Louis, MO Section of Hepatology, Department of Pediatrics, Washington University, St Louis, MO.
authors.forename: Adeel S
authors.initials: AS
authors.lastname: Khan
authors.forename: Brittany
authors.initials: B
authors.lastname: Brecklin
authors.forename: Neeta
authors.initials: N
authors.lastname: Vachharajani
authors.forename: Vijay
authors.initials: V
authors.lastname: Subramanian
authors.forename: Michelle
authors.initials: M
authors.lastname: Nadler
authors.forename: Janice
authors.initials: J
authors.lastname: Stoll
authors.forename: Yumirle
authors.initials: Y
authors.lastname: Turmelle
authors.forename: Jeffery A
authors.initials: JA
authors.lastname: Lowell
authors.forename: William C
authors.initials: WC
authors.lastname: Chapman
authors.forename: Majella Mb
authors.initials: MM
authors.lastname: Doyle
mesh_terms: D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D018197:Hepatoblastoma / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000473:pathology* / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome