title: Surgery of liver tumors in children in the last 15 years.
abstract: OBJECTIVE
  Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years.
  
  
  METHODS
  A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 +/- 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves.
  
  
  RESULTS
  Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results.
  
  
  CONCLUSIONS
  Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.
authors: Department of Pediatric Surgery, Transplantation Unit, Hospital Universitario La Paz, Madrid, Spain.
authors: A M
authors: AM
authors: Andres
authors: F
authors: F
authors: Hernandez
authors: M
authors: M
authors: Lopez-Santamaría
authors: M
authors: M
authors: Gámez
authors: J
authors: J
authors: Murcia
authors: N
authors: N
authors: Leal
authors: J C
authors: JC
authors: López Gutierrez
authors: E
authors: E
authors: Frauca
authors: A
authors: A
authors: Sastre
authors: J A
authors: JA
authors: Tovar
mesh_terms: D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000706:statistics & numerical data*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome