Surgery of liver tumors in children in the last 15 years.
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.
Andres
A M
AM
Department of Pediatric Surgery, Transplantation Unit, Hospital Universitario La Paz, Madrid, Spain.
Hernandez
F
F
Lopez-Santamaría
M
M
Gámez
M
M
Murcia
J
J
Leal
N
N
López Gutierrez
J C
JC
Frauca
E
E
Sastre
A
A
Tovar
J A
JA
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