Treatment outcomes for hepatoblastoma: an institution's experience over two decades.

Our objective is to review a leading Australian tertiary referral centre's experience in treating hepatoblastoma over two decades. This is a retrospective study of clinical, radiological and pathological data in hepatoblastoma patients treated at our institution between 1984 and 2004. Thirty children (17 male, 13 female) ranging in age from 5 months to 6.5 years (median 19 months) at diagnosis were reviewed. This is the largest case series reported in Australia to date. The median length of follow-up was 6.3 years (range 7 months to 20.9 years). On average, the number of new cases increased by 0.12 each year (P=0.01). A total of 29 patients underwent definitive surgery for primary tumour control. Of these, two (7%) had initial primary surgery, while 27 (93%) had delayed surgery following neo-adjuvant chemotherapy. Chemotherapy regimens included the SIOPEL study protocols. Patients not enrolled in SIOPEL tended to be given more courses of chemotherapy. Ten patients (34%) received an extended right hemi-hepatectomy, six (21%) had right hemi-hepatectomy, seven (24%) had left hemi-hepatectomy, three (10%) had left lateral segmentectomy, and two (7%) had a non-anatomical resection. In addition, two patients required IVC reconstruction at the time of their primary liver resection. Overall survival at 5 years was 89.1% (95% CI 69.8-96.4%). Event-free survival at 5 years was 75.7% (95% CI 53.2-88.5%). There was a clear increase in the risk of recurrence (RR=4.8) and death (RR=4.5) where margins were not microscopically clear. However, neither reached statistical significance in this small cohort. Surgery was still worthwhile even when margins were involved. There was no correlation between mean AFP level at diagnosis, and outcome. Our experience suggests that the achievement of microscopically clear margins at the time of primary tumour resection is an important factor in achieving a positive long-term outcome. In addition, extended courses of neo-adjuvant chemotherapy in patients with pulmonary metastases achieves good long-term outcomes, provided adequate surgical resection of the primary tumour with clear microscopic margins can be achieved.