F. Lopez-Verdugo1, A. Florez-Huidobro Martinez2, S. Fujita1, K. Jensen1, I. Zendejas1, E. R. Scaife1, L. Book1, R. L. Meyers1, M. I. Rodriguez-Davalos1 1Intermountain Primary Children’s Hospital,Department of Surgery, Transplant And Hepatobiliary Surgery,Salt Lake City, UT, USA 2Universidad Anáhuac,School Of Medicine,Mexico City, MX, Mexico
Introduction: Primary resection remains the mainstay treatment for liver malignancies in the pediatric population, unfortunately many of these children present with unresectable disease, for which liver transplantation has become the standard of care. Hepatoblastoma (HBL) is the most common type of liver malignancy in children, representing 80% of all liver tumors. It usually presents before the age of four and seems to affect more male patients with a ratio that varies from 1.2 to 3.6:1. The aim of this study is to review patient and graft survival in a cohort of patients with liver malignancy who underwent liver transplantation at our center over the past 2 decades and compare the different types of liver allografts.
Methods: All patients diagnosed with liver malignancy who underwent liver transplantation as treatment from 1998 to 2018 were analyzed. Demographics, age at the time of transplant, prior resections, type of graft, vascular complications, survival rate and recurrence were evaluated. Fisher’s exact test was performed to demonstrate differences in survival rate at 12-month follow-up between graft types used.
Results: From the 249 transplants performed in our center over the last two decades, 16 transplants (6.4%) were performed for malignancies in 15 patients. The mean age at transplant was 4.19 years (range: 0.6-7.1 years), 9 patients were female (60%). 13 transplants (81.2%) were performed for HBL, 2 for hemangioendothelioma (12.5%) and 1 for pancreatoblastoma (6.25%). 5 transplants were from living donors and 11 from deceased donors (3 reduced/split and 8 whole), 1 patient received an ABO incompatible liver. Half of our cohort received technical variants grafts either from living or deceased donors. Out of the patients with HBL, 4 (30%) had a prior resection attempt; among these, 2 patients (50%) succumbed within 2 months after the LTx. Overall, 3 patients died within the first 6 months after LTx. Causes of death included recurrence of the disease (n=2) and primary graft non-function (n=1). All the patients with diseases other than HBL are alive and doing well. At a median follow-up of 84.5 months (range: 0-241) overall patient and graft survival were 80% and 75%, respectively. There was no statistically significant difference on survival rate between patients who received whole grafts compared to those who received technically variant grafts (p=0.5).
Conclusion:Timing is critical in providing liver transplantation for patients suffering from liver malignancy in which extent of the disease precludes complete resection. The use of segmental grafts and ABOi livers did not appear to diminish survival rate at 1 year follow-up; thus, utilization of these graft types might increase the organ donor pool and expedite treatment for patients with liver malignancy. The most important factor in our series was histology of the tumor as this was consistent with small cell undifferentiated variant of HBL in patients that died of recurrence of disease.