Liver transplantation for unresectable high risk & very high risk hepatoblastoma

Abstract

Introduction: Hepatoblastoma is the most common childhood primary hepatic malignancy, with an incidence of 1:1,000,000. Treatment consists of neoadjuvant chemotherapy follow by resection or transplantation, and adjuvant chemotherapy. Our goal is to evaluate liver transplantation for unresectable high risk and very high risk hepatoblastoma patients. Methods: A retrospective review of 42 consecutive pediatric liver transplants at our center from March 1, 2013 to Aug 31, 2017. Patients were evaluated using the PRETEXT guidelines and the CHIC risk stratifications score. Liver function tests including alpha feto protein (AFP) were tested. All patients underwent liver biopsy at the time of diagnosis. POSTEXT was evaluated based on last abdominal imaging before transplant. Explant pathology was reviewed. Results: A total of 8 patients underwent transplantation for hepatoblastoma, 4 were PRETEXT III and 4 were PRETEXT IV. Based on imaging concerns for vascular involvement 5 cases were deemed high risk, the 3 remaining cases were deemed very high risk based on presence of lymph nodes or pulmonary metastases. Tumor was multifocal in 3 cases. All patients received deceased donor grafts, 6 whole grafts and 2 split grafts. All patients had elevated AFP (>100 ng/ml). Pre-treatment biopsies showed epithelial fetal histology in 4 (50%) cases, the remaining 4 (50%) cases were epithelial mixed fetal and embryonal. Median follow up was 26 months, no cases with recurrence. In our study there was no correlation with prematurity or low birth weight. Conclusion: In our experience, excellent outcomes can be achieved for unresectable high risk and very high risk hepatoblastoma patients.