Definitive surgical outcomes in children with advanced hepatoblastoma (PRETEXT III-IV): A retrospective single-center Canadian experience.

Abstract

Children with advanced hepatoblastoma (PRETEXT III-IV) often require complex surgical decision-making; however, contemporary data describing long-term outcomes and recurrence patterns across the full spectrum of definitive surgery remain limited. We included 68 patients with PRETEXT III and IV who underwent definitive liver resection (LR, n=41) or liver transplantation (LT, n=27). The primary objective of this study was to characterize survival and recurrence patterns after surgical clearance of primary liver disease. The median age at diagnosis was 18 months (IQR, 9-36). All the patients received neoadjuvant chemotherapy. With a median follow-up of 62 months (IQR, 25-146), 5-year overall survival was 79% (95% CI: 65%-97%) after LT and 88% (95% CI 78%-100%) after LR. After LT, recurrence occurred in 6 of the 27 (22%) patients, including isolated lung metastases (n=4), combined lung-graft involvement (n=1), and isolated graft relapse (n=1). Recurrence occurred in 6 of the 41 patients (15%) after LR, involving the liver (n=2), lung (n=2), combined liver and pulmonary disease progression (n=1), and unspecified site (n=1). Among LR patients with residual pulmonary nodules at surgery (n=8), progression occurred in 3, 2 of whom died. The majority of events occurred within the first postoperative year after LR or LT. All deaths (n=10, 15%) were attributable to tumor recurrence or progression of known lung metastasis, including all post-LT recurrences. There were no perioperative or graft-related deaths. Univariate analysis showed that a serum alpha-fetoprotein decline of <95% and distant metastasis at diagnosis were associated with poor prognosis after LT and LR. Mortality in this cohort was entirely disease-related, occurring after recurrence or progression of residual metastases rather than from surgical modality. Incorporating AFP trend, pulmonary response, and detailed imaging into preoperative assessment may help identify high-risk patients and support more informed surgical decision-making.