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

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

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  • 1Department 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.

PMID:
10466608
[PubMed - indexed for MEDLINE]
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