Survival after liver transplantation for hepatoblastoma

a 2-center experience

Marybeth Browne, Dani Sher, David Grant, Enza Deluca, Estella M Alonso, Peter F Whitington, Riccardo A Superina*

*Corresponding author for this work

Research output: Contribution to journalArticle

51 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1973-1981
Number of pages9
JournalJournal of Pediatric Surgery
Volume43
Issue number11
DOIs
StatePublished - Nov 1 2008

Fingerprint

Hepatoblastoma
Liver Transplantation
Drug Therapy
Survival
Neoplasms
Fetal Proteins
Transplants
Adjuvant Chemotherapy
Blood Vessels
Histology
Survival Rate
Transplantation
Recurrence
Therapeutics

Keywords

  • Children
  • Hepatoblastoma
  • Liver transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

@article{c3bded69850e462b8d4242f4ca46a75f,
title = "Survival after liver transplantation for hepatoblastoma: a 2-center experience",
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.",
keywords = "Children, Hepatoblastoma, Liver transplantation",
author = "Marybeth Browne and Dani Sher and David Grant and Enza Deluca and Alonso, {Estella M} and Whitington, {Peter F} and Superina, {Riccardo A}",
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Survival after liver transplantation for hepatoblastoma : a 2-center experience. / Browne, Marybeth; Sher, Dani; Grant, David; Deluca, Enza; Alonso, Estella M; Whitington, Peter F; Superina, Riccardo A.

In: Journal of Pediatric Surgery, Vol. 43, No. 11, 01.11.2008, p. 1973-1981.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Survival after liver transplantation for hepatoblastoma

T2 - a 2-center experience

AU - Browne, Marybeth

AU - Sher, Dani

AU - Grant, David

AU - Deluca, Enza

AU - Alonso, Estella M

AU - Whitington, Peter F

AU - Superina, Riccardo A

PY - 2008/11/1

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N2 - 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.

AB - 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.

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KW - Hepatoblastoma

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