Surgical Management and Outcomes of Relapsed and Refractory Hepatoblastoma: An Institutional Case Series.
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Relapse occurs in 10%-20% of children with hepatoblastoma and is associated with inferior outcomes, yet standardized management remains limited. Defining risk factors and relapse patterns, as well as the role of surgery, particularly transplantation, is critical to optimizing survival. A retrospective review was performed of patients aged 0-18 years diagnosed with hepatoblastoma from 2007 to 2024. Demographics, tumor characteristics, treatment course, relapse patterns, and outcomes were collected. Analyses included univariable and multivariable logistic regression, Kaplan-Meier survival estimation, and log-rank testing. Among 135 patients, 98 (72.6%) achieved remission without relapse, 32 (23.7%) relapsed, and 5 (3.7%) experienced primary progression without remission. Older age, PRETEXT (pretreatment extent of disease) IV, metastatic disease at diagnosis, and macrovascular invasion were associated with relapse on univariable analysis, but only older age remained independently associated with relapse (adjusted odds ratio 1.27 per year, 95% confidence interval 1.05-1.53); PRETEXT IV and metastatic disease were not significantly associated with overall survival. Lung-only relapse demonstrated favorable survival (100% alive), whereas combined liver and lung or extrahepatic relapse had poorer outcomes (40%-44% alive), although survival did not differ significantly across relapse sites (p = 0.15). Of 54 liver transplant recipients, 44 underwent upfront transplantation, and 10 underwent salvage transplantation, with no significant difference in overall survival (p = 0.90), although the salvage group was small. Relapse or progression after upfront transplantation occurred in 27% of recipients; lung-only relapse again conferred favorable survival. Long-term survival is achievable in relapsed hepatoblastoma, particularly for isolated pulmonary relapse. Older age, likely a surrogate for adverse tumor biology, was associated with relapse but did not preclude survival when aggressive multimodal therapy was used. Salvage transplantation achieved durable survival in selected patients and should be considered for unresectable intrahepatic relapse when extrahepatic disease is absent or controlled.