Surgical Management and Outcomes of Relapsed and Refractory Hepatoblastoma: An Institutional Case Series.
9 affiliations
- 1Department of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
- 2Department of Surgery, University of Cincinnati Medical Center, Cincinnati, Ohio, USA.
- 3Department of Surgery, Brigham and Women's Hospital, Boston, Massachusetts, USA.
- 4Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
- 5Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
- 6Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
- 7Department of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
- 8Peckham Center for Cancer and Blood Disorders, Rady Children's Hospital, San Diego, California, USA.
- 9Division of Abdominal Transplantation, Stanford Medicine Children's Health, Palo Alto, California, USA.
Abstract
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.
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BibTeX
@article{ray2026surgical,
title = {{Surgical Management and Outcomes of Relapsed and Refractory Hepatoblastoma: An Institutional Case Series.}},
author = {Marissa Ray and Alyssa Stetson and Nicolas Noriega and Stephen Hartman and Colton Brown and Emily Schepers and Zishaan Farooqui and Alexander J Towbin and Katherine M Somers and Sarangarajan Ranganathan and James Geller and Sue Evans and Jonathan Merola and Alexander J Bondoc and Greg Tiao},
journal = {Cancer medicine},
year = {2026},
month = oct,
doi = {10.1002/cam4.72453},
url = {https://europepmc.org/article/MED/42958585},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Surgical Management and Outcomes of Relapsed and Refractory Hepatoblastoma: An Institutional Case Series.
AU - Marissa Ray
AU - Alyssa Stetson
AU - Nicolas Noriega
AU - Stephen Hartman
AU - Colton Brown
AU - Emily Schepers
AU - Zishaan Farooqui
AU - Alexander J Towbin
AU - Katherine M Somers
AU - Sarangarajan Ranganathan
AU - James Geller
AU - Sue Evans
AU - Jonathan Merola
AU - Alexander J Bondoc
AU - Greg Tiao
PY - 2026
DA - 2026/10/29
JO - Cancer medicine
DO - 10.1002/cam4.72453
UR - https://europepmc.org/article/MED/42958585
AB - 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.
SN - 2045-7634
ER -