Predictors of survival and incidence of hepatoblastoma in the paediatric population

Division of General Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
HPB (Impact Factor: 2.68). 04/2013; 15(10). DOI: 10.1111/hpb.12112
Source: PubMed


This study evaluates current trends in incidence, clinical outcomes and factors predictive of survival in children with hepatoblastoma (HB).

The Surveillance, Epidemiology and End Results (SEER) database was queried for the period 1973-2009 for all patients aged <20 years with HB.

A total of 606 patients were identified. The age-adjusted incidence was 0.13 patients per 100 000 in 2009. An annual percentage change of 2.18% (95% confidence interval (CI) 1.10-3.27; P < 0.05) was seen over the study period. Overall survival rates at 5, 10 and 20 years were 63%, 61% and 59%, respectively. Ten-year survival rates significantly improved in patients with resectable disease who underwent operative treatment in comparison with those with non-resectable HB (86% versus 39%; P < 0.0001). Multivariate analysis showed surgical treatment (hazard ratio (HR) = 0.23, 95% CI 0.17-0.31; P < 0.0001), Hispanic ethnicity (HR = 0.61, 95% CI 0.43-0.89; P = 0.01), local disease at presentation (HR = 0.43, 95% CI 0.29-0.63; P < 0.0001) and age < 5 years (HR = 0.63, 95% CI 0.41-0.95; P < 0.03) to be independent prognostic factors of survival.

The incidence of paediatric HB has increased over time. Hepatoblastoma is almost exclusively seen in children aged < 5 years. When HB presents after the age of 5 years, the prognosis is most unfavourable. Tumour extirpation markedly improves survival in paediatric patients with local disease.

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    • "Hepatoblastoma, the most common pediatric hepatic malignancy, accounts for about 50% of malignant liver tumors in children [1]. More than half of the children presenting with hepatoblastoma have advanced or metastatic disease at the time of diagnosis [2], and survival remains poor for those with unresectable or recurrent disease [3]. Malignant rhabdoid kidney tumors (MRKT) are another type of aggressive pediatric solid tumor. "
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