Elsevier

Journal of Pediatric Surgery

Volume 43, Issue 11, November 2008, Pages 1973-1981
Journal of Pediatric Surgery

Survival after liver transplantation for hepatoblastoma: a 2-center experience

https://doi.org/10.1016/j.jpedsurg.2008.05.031Get rights and content

Abstract

Purpose

Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy.

Methods

All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, α-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels.

Results

Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 ± 48 months). Patients were transplanted a mean of 4 ± 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival.

Conclusions

Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.

Section snippets

Materials and methods

All patients who were transplanted for unresectable hepatoblastoma at 2 different institutions between the years of 1990 and 2004 were included in this analysis. A retrospective review of their records was performed. The variables that were analyzed included age, sex, previous tumor resection, pretransplantation α-fetoprotein (AFP) response to chemotherapy, metastatic disease at diagnosis, histological vascular invasion, tumor histology, and administration of posttransplantation chemotherapy. α

Results

Fourteen patients received 15 transplants for hepatoblastoma. One was retransplanted for hepatic artery thrombosis 10 days after the first transplant. All patients received neoadjuvant chemotherapy, and 5 (36%) received adjuvant chemotherapy after the transplant. Table 1 outlines the agents received, the number of cycles, and the PRETEXT staging before transplantation. After diagnosis, each child completed at least 2 cycles of neoadjuvant chemotherapy before being considered for transplantation

Discussion

It is possible in most children with hepatoblastoma to completely resect the tumor without transplantation. However, even with aggressive contemporary chemotherapy, a small but significant number of patients, because of location or size or multicentricity of the tumor, can only have the tumor adequately excised by a complete hepatectomy For these children, LT offers the only hope for cure. Liver transplantation is indicated for children whose tumors cannot be resected without leaving them with

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    Studies conducted at different centers worldwide also show a trend toward better results with hepatectomy vs transplant. However, comparisons lack statistical significance because of small sample sizes [18–21]. We believe that aggressive hepatectomy and avoidance of transplant is a viable option with short- and long-term benefits for patients with hepatoblastoma.

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