title: Review of outcomes of primary liver cancers in children: our institutional experience with resection and transplantation.
abstract: BACKGROUND
  Operative intervention plays an important role in the management of primary liver cancers in children. Recent improvements in diagnostic modalities, pre- and postoperative chemotherapy, and operative technique have all led to improved survival in these patients. Both hepatic resection and orthotopic liver transplantation are effective operations for pediatric liver tumors; which intervention is pursued is based on preoperative extent of disease. This is a review of our institution's experience with operative management of pediatric liver cancer over an 18-year period.
  
  
  METHODS
  A retrospective chart review from 1990 to 2007 identified patients who were ≤18 years old who underwent operative intervention for primary liver cancer. Demographics, type of operation, intraoperative details, pre- and postoperative management, as well as outcomes were recorded for all patients.
  
  
  RESULTS
  Fifty-four patients underwent 57 operations for primary liver cancer, 30 of whom underwent resection; the remaining 27 underwent orthotopic liver transplantation. The mean age at diagnosis was 41 months. Twenty patients had stage 1 or 2 disease and 34 patients had stage 3 or 4 disease. Forty-eight (89%) patients received preoperative chemotherapy. Postoperative chemotherapy was given to 92% of patients. Mean overall and intensive care unit duration of stay were 18 and 6 days, respectively. About 45% of patients had a postoperative complication, including hepatic artery thrombosis (n = 8), line sepsis (n = 6), mild acute rejection (n = 3), biliary stricture (n = 2), pneumothorax (n = 2), incarcerated omentum (n = 1), Horner's syndrome (n = 1), and urosepsis (n = 1). Only 6 patients had a recurrence of their cancer, 5 after liver resection, 3 of whom later received a transplant. There was only 1 recurrence after liver transplantation. There was 1 perioperative mortality from cardiac arrest. Overall survival was 93%.
  
  
  CONCLUSIONS
  Operative intervention plays a critical role in the management of primary liver cancer in the pediatric population. Neoadjuvant chemotherapy can be given if the tumor seems unresectable at diagnosis. If chemotherapy is unable to sufficiently downstage the tumor, orthotopic liver transplantation becomes the patient's best option. Our institution has had considerable experience with both resection and liver transplantation in the treatment of pediatric primary liver cancer, with good long-term outcomes.
authors: Division of Pediatric General and Thoracic Surgery, University of Pittsburgh Medical Center, Children's Hospital of UPMC, Pittsburgh, PA 15224, USA.
authors: Marcus M
authors: MM
authors: Malek
authors: Sohail R
authors: SR
authors: Shah
authors: Prashant
authors: P
authors: Atri
authors: Jose L
authors: JL
authors: Paredes
authors: Leigh Anne
authors: LA
authors: DiCicco
authors: Rakesh
authors: R
authors: Sindhi
authors: Kyle A
authors: KA
authors: Soltys
authors: George V
authors: GV
authors: Mazariegos
authors: Timothy D
authors: TD
authors: Kane
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D006528:Carcinoma, Hepatocellular / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D020360:Neoadjuvant Therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents