Liver Transplantation for the Management of Hepatoblastoma U. Cillo, F.A. Ciarleglio, M. Bassanello, A. Brolese, A. Vitale, P. Boccagni, G. Zanus, L. Zancan, L. D’Antiga, P. Dall’igna, U. Montin, E. Gringeri, A. Carraro, G. Cappuzzo, P. Violi, M. Baldessin, A. Bridda, D. F. D’Amico, and G. Perilongo ABSTRACT Introduction. Hepatoblastoma (HEP) is the most frequent liver malignancy occurring in childhood. Surgical resection currently represents the gold standard for treatment. In patients with initially unresectable tumors, chemotherapy may induce remarkable reduc- tions in size. In nonresponder patients, liver transplantation (OLTx) may offer a chance of cure. Materials and Methods. From 1990 to 2003, a total of 400 OLTx (31 pediatric transplants) have been performed at Padua University. Seven patients (4 males and 3 females) underwent OLTx for hepatoblastoma. All patients presented with bilobar liver involvement and had received chemotherapy according to the SIOPEL-1. In all patients preoperative staging was negative for extrahepatic involvement. Results. The mean age of the pts was 8.2 years (range 6.4 months to 34 years). Mean follow-up after OLTx was 41.4 months (median 36, range 3 to 108 months). Actuarial patient survival rates after OLTx for hepatoblastoma are 83.3%, 83.3%, and 56% at 1, 3, and 5 years, respectively. Five of seven subjects with HEP are alive after transplant at 3, 12, 36, 65, and 108 months. Two patients died owing to recurrent disease after 6 and 60 months, respectively, from transplantation. Another subject, primarily treated with surgical resection, shows HEP recurrence at 40 months after OLTx. The remaining 4 patients are alive and well at a mean follow-up of 28 months (median 24, range 3 to 65 months). Conclusions. Liver transplantation may represent a valid therapeutic option for patients with unresectable HEP, but it is contraindicated in cases of recurrence following previous resection surgery. Neo-adjuvant chemotherapy is of paramount importance to obtain good long-term results. H EPATOBLASTOMA (HEP) is the most common primary liver tumor in children, accounting for just over 1% of pediatric cancers. 1 The etiology is unknown, but it has been associated with Beckwith-Weidemann syndrome, familia adenomatous polyposis, and low birth weight. 2,3 The prognosis is dependent upon resectability: it has been accepted that complete surgical removal of tumor is essential for a cure. Without complete resection the prognosis is poor. 4,5 Total hepatectomy and liver transplantation (LTx) has become a rational method for tumors that are unresectable after preoperative adjuvant chemotherapy. Malignant unresectable disease confined to the liver is considered to be a good indication for transplantation. The purpose of this study was to deter- mine the outcome of a population with a diagnosis of unresectable HEP who underwent liver transplantation as a curative modality. MATERIALS AND METHODS The 400 liver transplantations performed between November 1990 and March 2003 included 31 children (7.75%). During this period seven patients were transplanted with a diagnosis of hepatoblas- From Clinica Chirurgica and Liver Transplantation Unit, Padua University, Padua, Italy. Address reprint requests to Marco Bassanello, MD, Clinica Chirurgica 1i and Liver Transplantation Unit, “P.G. Cevese” Department of Surgical and Gastroenterlogical Sciences, Via Giustiniani 2, Padova 35128, Italy. E-mail: bassanellomarco @hotmail.com © 2003 by Elsevier Inc. All rights reserved. 0041-1345/03/$–see front matter 360 Park Avenue South, New York, NY 10010-1710 doi:10.1016/j.transproceed.2003.10.072 Transplantation Proceedings, 35, 2983-2985 (2003) 2983