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DOI: 10.1055/s-0030-1262798
© Georg Thieme Verlag KG Stuttgart · New York
High-Risk Hepatoblastoma: Results in a Pediatric Liver Transplantation Center
Publication History
received May 18, 2010
accepted after revision June 18, 2010
Publication Date:
11 October 2010 (online)
Abstract
Aim: Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available.
Methods: 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and <100 ng/ml serum AFP) were identified and imaging and biopsies were reviewed. The treatment consisted of total removal of the tumor, involving extended hepatectomies and LT if necessary, together with SIOPEL-guided chemotherapy.
Results: 23/57 hepatoblastomas were HRH (11F/12M). 17 were considered unresectable by standard techniques, 3 had extrahepatic disease, and 3 fulfilled both criteria. Mean age at diagnosis was 2.3±2.4 years. 3 children (referred after chemotheraphy) died without surgery. 4 had resections (2 left and 2 right trisegmentectomies). Primary LT was required in 15 children (7 cadaveric donors and 8 living related donor transplantations (LRDT), 2 of them with retrohepatic vena cava replacement), and 1 patient had rescue LT after recurrence. Mean follow-up was 4.8±2.9 years. 2 children who had undergone liver resection developed pulmonary metastases at 1.7 and 1.6 years postoperatively and survived after surgical treatment. 2 children with LT developed EBV-related lymphoma and leukemia respectively but survived. Event-free survival (EFS) at 1, 5, and 10 years was 78.3±8.6%, 63.1±10.5%, and 63.1±10.5%, respectively. 6 children died (3 without surgery, 1 after liver resection, 1 after primary LT and 1 after rescue LT). Overall survival at 1, 5 and 10 years was 78.3±21.7%, 73.2±26.8% and 73.2±26.8%. Of those with primary LT, survival at 1, 5 and 10 years was 93.3±6.4%, 93.3±6.4% and 93.3±6.4%.
Conclusions: Outstanding results in the treatment of HRH are possible in tertiary centers when referral is early (preferably at diagnosis) and specialized liver surgery and transplantation facilities are available.
Key words
hepatoblastoma - transplantation - high risk - resection
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Correspondence
Dr. S. Barrena
Hospital Universitario La Paz
Cirugía Pediátrica
Paseo de la Castellana
28046 Madrid
Spain
Phone: +34 91 7277 019
Fax: +37 91 7277 478
Email: sbarrena@hotmail.com