Treatment outcomes for hepatoblastoma: experience of 35 cases at a single institution.

BACKGROUND/PURPOSE: Hepatoblastoma is the most common malignant liver tumor in children. Comparative studies have elucidated the optimal pre- or postoperative chemotherapeutic regimens. The aim of this study was to investigate the prognostic significance of baseline tumor characteristics for overall survival and disease-free survival in children with hepatoblastoma.

METHODS

There were 19 male and 16 female children with a median age of 19 months at diagnosis (range: 1-169 months) in our institution between February 1990 and June 2009. We reviewed the clinical presentation, serum α-fetoprotein level at diagnosis, histological subtype, treatment, and outcomes.

RESULTS

Twenty-seven patients (78%) underwent neoadjuvant chemotherapy. The majority of patients subsequently underwent either hemihepatectomy (56%) or bisegmentectomy (16%). Only six patients underwent extended hepatic resection, and one of them required rescue liver transplantation. During follow-up, six patients died of progressive disease and two of perioperative mortality. Four of the six who died had pulmonary metastases at the time of diagnosis or follow-up. The median survival time was 28 months (range: 1-181 months). Five-year overall survival was 67.7% (95% confidence interval: 52.0-87.8%) and disease-free survival was 60.2% (95% confidence interval: 41.9-86.5%).

CONCLUSION

The potential down-staging effect of neoadjuvant chemotherapy on hepatoblastoma might facilitate remission and convert unresectable tumors into operable ones.