INTRODUCTION
Hepatoblastoma (HB) is the most common primary hepatic neoplasm in children. The mainstays of HB treatment are surgical resection and cisplatin-doxorubicin (PLADO) chemotherapy (Qtx). If the tumor is considered unresectable at diagnosis, neoadjuvant chemotherapy can make the lesion resectable in up to 80% of the patients1. For patients with PRETEXT III or IV that remain unresectable after neoadjuvant Qtx, and do not present distant metastasis, liver transplantation (LT) is the preferred treatment alternative. Current 10-year post-LT overall survival for unresectable HB is over 80%2.
Although patients with HB receive exception points on the waiting list to compete with other children with end-stage liver disease for a liver graft, the timing to perform the LT is crucial for a better outcome. Usually these patients have an optimal treatment window – after completion of the Qtx – when the transplant can be performed. The importance of living donor liver transplantation (LDLT) in this context has been previously described3, 4. The majority of the reports on LDLT for HB are based on a limited number of cases, and most studies show recurrence rates from 20% to 37.5%4-6.
It is well known that response to chemotherapy, manifested as either a decrease in tumor size or a significant decrease in alpha-fetoprotein (AFP) level, is the most important prognostic factor for successful LT7. However, lengthy courses of preoperative chemotherapy while the tumor remains unresectable should be avoided due to diminishing effects on the tumor, combined with the substantial risk of inducing Qtx resistance with the prolonged exposure 8.
The ideal scenario when treating patients with unresectable HB should include response to Qtx and the availability of liver graft for transplantation. However, how far to push the transplant indication in patients with a marginal response to chemotherapy (but no distant metastasis) and with an available living donor? In the present study, the outcome of 28 patients who received LDLT is reported with emphasis in the factors associated with recurrence/death after transplantation.