This manuscript was originally submitted to and accepted for publication in Medical & Pediatric Oncology by its Editor-in-Chief, Dr. G. D'Angio.
Liver transplantation for hepatoblastoma: Results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience†
Article first published online: 25 NOV 2003
Copyright © 2003 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 42, Issue 1, pages 74–83, January 2004
How to Cite
Otte, J.B., Pritchard, J., Aronson, D.C., Brown, J., Czauderna, P., Maibach, R., Perilongo, G., Shafford, E. and Plaschkes, J. (2004), Liver transplantation for hepatoblastoma: Results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience. Pediatr. Blood Cancer, 42: 74–83. doi: 10.1002/pbc.10376
- Issue published online: 25 NOV 2003
- Article first published online: 25 NOV 2003
- Manuscript Accepted: 25 FEB 2003
- Manuscript Received: 29 MAY 2002
- The Cancer Research Campaign (United Kingdom)
- the Swiss Cancer League (Berne)
- the Children's Liver Tumor Parents' organisation (United Kingdom)
- childhood cancer;
- liver malignancies;
- liver transplantation
For hepatoblastoma (HB) that remains unresectable by partial hepatectomy after chemotherapy, total hepatectomy with orthotopic liver transplantation (LTX) has been advocated as the best treatment option. The role of LTX in the overall management of HB is still, however, unclear.
The results of LTX from the first study of HB by the International Society of Pediatric Oncology, SIOPEL-1, were analyzed. In addition, the world experience of LTX for HB was extensively reviewed. Twelve patients in the SIOPEL-1 study underwent a LTX. Median (range) follow-up at Dec. 31, 2001 was 117 months (52–125) since LTX.
Overall survival at 10 years post-LTX was 85% for the seven children who received a “primary LTX” and 40% for the 5 children who underwent a “rescue LTX” after previous partial hepatectomy. In the world experience (147 cases), the overall survival rate at 6 year post-LTX was 82% for 106 patients who received a “primary LTX” and 30% for 41 patients who underwent a “rescue LTX.” Multivariate analysis of patients undergoing primary LTX showed that only macroscopic venous invasion had a significant impact (P-value: 0.045 with a hazard ratio of 2.96) on overall survival.
Orthotopic LTX has added a new dimension to the treatment of HB unresectable by partial hepatectomy. Because of the rarity of the disease and to optimize results, children with extensive HB should be treated in centers with surgical expertise in pediatric major liver resection and LTX, in close collaboration with pediatric oncologists, radiologists, and histopathologists. Pediatr Blood Cancer 2004;42:74–83. © 2003 Wiley-Liss, Inc.