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

Authors

Sagar Mehta, Blayne Amir Sayed, Furqan Shaikh, Fernanda Takamatsu, Eveline Lapidus-Krol, Or Steg Saban, Jennifer Stunguris, Maria De Angelis, Krista Van Roestel, Julia Hensley, Yaron Avitzur, Robert Hj Bandsma, Nicola Jones, Binita M Kamath, Justin T Gerstle, Anand Ghanekar, Mark Cattral, Vicky L Ng, Nicolas Goldaracena

Published in

Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. Sep 29, 2026. Epub Sep 29, 2026.

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 three, two 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 less than 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.

PMID:
42809825
Bibliographic data and abstract were imported from PubMed on 30 Sep 2026.

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