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.