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Excellent Outcome Following Liver Transplantation for Hepatoblastoma Using an Extensive En Bloc Hepatectomy Technique
Institution:1. Department of Visceral Transplant Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;2. Department of Paediatric Hepatology and Liver Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;3. Institute of Legal Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;1. Department of General Surgery, the First Affiliated Hospital of Nanchang University, Nanchang, China;2. Department of Critical Care Medicine, the First Affiliated Hospital of Nanchang University, Nanchang, China;3. Department of Hepatobiliary Surgery, the First Affiliated Hospital of Xi''an Jiaotong University, Xi''an, China;1. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan;2. Department of Kidney Disease Center, Nagoya Daini Red Hospital, Nagoya, Japan;1. Department of Translational and Precision Medicine, Nephrology Unit, Sapienza University of Rome, Rome, Italy;2. Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences, Sapienza University of Rome, Rome, Italy;3. Department of General Surgery, Organ Transplant Unit, Sapienza University of Rome, Rome, Italy;4. Clinical Pathology, Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy;5. Clinical Pathology, Department of Surgery \"P. Stefanini\", Sapienza University of Rome, Rome, Italy;1. Department of Anesthesiology, Intensive Care and Pain Medicine, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;2. Kanta-Häme Central Hospital, Hämeenlinna, Finland;3. Department of Anesthesia, Faculty of Medicine and Life Sciences, University of Tampere, Tampere, Finland;4. Transplantation and Liver Surgery Clinic, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;5. Children''s Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Abstract:BackgroundHepatoblastoma is a rare malignancy but the most common primary hepatic malignancy in childhood. Pediatric liver transplantation (LT) offers the possibility to achieve a complete resection in otherwise unresectable tumors. Almost no data are available regarding specific surgical technique of LT in children with hepatoblastoma.MethodsWe analyzed all children with hepatoblastoma and LT between 2007 and 2012. Special regard was given to the surgical technique and long-term follow-up.ResultsOverall 7 children were transplanted with the diagnosis of hepatoblastoma (5 male, 2 female). Thereof, 4 children (median age was 11 months, range, 6–31 months) underwent “primary” LT for hepatoblastoma Pretreatment Extent of Disease III to IV. A 4-year-old boy received “salvage” LT for recurrent hepatoblastoma 2.5 years after successful liver resection. Another 15-year-old boy was transplanted as a prophylactic treatment after repeated liver resection for hepatoblastoma due to the high recurrence risk. A 14-year-old boy underwent LT due to complications following liver resection for hepatoblastoma during infancy. In all children, extensive en bloc hepatectomy was performed together with resection of the adjoining retroperitoneal tissue and regional lymphadenectomy. Actually, all children are alive without tumor recurrence median 7.1 years after LT (range, 5.7–10.7 years).ConclusionOur data show an excellent long-term outcome in selected children with hepatoblastoma undergoing standardized en bloc hepatectomy for “primary” and “rescue” LT with 100% overall and recurrence-free survival.
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