J Pediatr Surg 1999 Aug;34(8):1261-4

Orthotopic liver transplantation for unresectable hepatoblastoma: a single
center's experience

Al-Qabandi W, Jenkinson HC, Buckels JA, Mayer AD, McKiernan P, Morland B, John
P, Kelly D

Department of Oncology, The Birmingham Children's Hospital NHS Trust, England

Abstract
BACKGROUND/PURPOSE: Complete surgical resection after chemotherapy is the
definitive treatment for hepatoblastoma. However, orthotopic liver
transplantation (OLT) is now accepted as a treatment modality for patients with
unresectable tumours. The aim of this study was to review a single center's
experience of OLT for unresectable hepatoblastoma. METHODS: A retrospective
review of 8 patients with unresectable hepatoblastoma who were referred for
liver transplantation was conducted. RESULTS: The patients assessed had an age
range of 5 to 105 months at presentation; median, 24 months, (5 boys; 3 girls).
Two patients have familial adenomatous polyposis, and one has right
hemihypertrophy. All 8 patients had received standard chemotherapy according to
SIOP (International Society of Pediatric Oncology) protocols. Extrahepatic
metastases were found in 3 patients at diagnosis, but none had detectable
metastases at the time of OLT. Four patients continued chemotherapy while
awaiting OLT. Three patients received whole grafts, and 5 received reduced
grafts. The median follow-up period was 22 months (range, 2 to 78 months). Five
patients are alive and well, although 1 patient had a second OLT for biliary
cirrhosis secondary to biliary stricture at 6 years. Three patients died: one 26
days post OLT of sepsis and two of disease recurrence at 22 months and 70 months
posttransplant. The actuarial survival rate is 88% and 65% at 1 and 5 years,
respectively, whereas the overall survival rate is 62.5%. CONCLUSION: OLT for
unresectable hepatoblastoma without extra hepatic metastases is highly
successful with a low recurrence rate