Survival trends in children with hepatoblastoma.

PURPOSE: Hepatoblastoma (HB) is a relatively rare pediatric malignancy. In this study, we present demographic data and a survival analysis from the largest patient cohort with HB reported to date.

METHODS

The surveillance, epidemiology, and end results database was queried from 1973 to 2005 for all patients diagnosed with HB. Kaplan-Meier survival analysis was conducted to determine actuarial survival. Cox regression analysis was performed to determine hazard ratios (HR) for prognostic variables.

RESULTS

During the study period, 459 patients with HB were identified. Overall 1-, 3-, and 5-year survival rates for the entire patient cohort were 76, 63, and 60%, respectively. Five-year survival improved over time from 36 (1973-1982) to 63% (1983-2005). Predictors of poor survival include: age 2 years and greater (HR 1.566), black race (HR 1.910), diagnosis prior to 1983 (HR 3.327), inability to perform surgical resection (HR 3.857), regional disease (HR 1.939), and distant disease (HR 3.196).

CONCLUSIONS

Hepatoblastoma continues to challenge surgeons and oncologists. Most children are diagnosed early in life and undergo surgical resection whenever possible. With the advent of efficacious chemotherapy, survival has improved. Older children, black patients, and those who present with advanced disease tend to have poor outcomes. Surgical resection is the single most important predictor of survival.