Author information
1
Hannover Medical School, Div of Pediatric Gastroenterology and Hepatology, Department for Pediatric Kidney-, Liver- and Metabolic Diseases, Hannover, Germany.
2
European Liver Transplant Registry, APHP Hôpital Paul Brousse, Université Paris-Sud, Inserm U 935, Villejuif, France.
3
Department of Hepatology and Liver Transplant Unit, Henri Mondor Hospital APHP, Paris Est University, Créteil, France.
4
Research Group Epidemiological and Statistical Methods, Helmholtz Centre for Infection Research4, Braunschweig, Germany.
5
German Center for Infection Research (DZIF), Hannover-Braunschweig site.
6
Pediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Italy.
7
Hopital Necker Enfants Malades, Service de Chirurgie Pediatrique, Paris, France.
8
Department of Surgery Division, Division of Hepatobiliary and Livertransplantation, Ege University Medical School, Izmir.
9
Papa Giovanni 23 Hospital, Chirurgia III e Centro Trapianti di Fegato, Bergamo, Italy.
10
Department of Transplantation Surgery, Huddinge Hospital, Huddinge, Sweden.
11
Rikshospitalet, Radiumhospitalet Medical Center Liver Transplant Unit, Oslo, Norway.
12
King's College London, London, England.
13
U.C.Helsingfors, Transplantation and Liver Surgery Clinic, Helsinki, Finland.
14
Hannover Medical School, Klinik für Viszeral und Transplantationschirurgie, Hannover, Germany.
15
Cliniques Universitaires Saint Luc, Catholic University Of Louvain, Brussels, Belgium.
16
Children's Hospital Pittsburgh, Pittsburgh, United States Of America.
17
Swiss Center for Liver Disease in Children, Hôpitaux Universitaires de Genève, Genève, Switzerland.
18
C.U.K. GHS Essen, Klinik für allgemeine und Transplantationschirurgie, Essen, Germany.
19
Centro De Trapianti de Fegato, Azienda Ospedaliera S. Giovanni Battista, Torino, Italy.
20
Hospitais da Universidade de Coimbra, Gabinete de Coordenacao de Colheita de Orgaos e Transplantacao, Coimbra, Portugal.
21
University Hospital, Dept. Of General & Transplant Surgery, Innsbruck, Austria.
Abstract
Hepatocellular carcinoma (HCC) in childhood differs from adult HCC as it is often associated with inherited liver disease. It is, however, unclear whether liver transplantation (LT) for HCC in childhood with or without associated inherited disease has a comparable outcome to adult HCC. Based on data from the European Liver Transplant Registry (ELTR) we aimed to investigate if there are differences in patient and graft survival after LT for HCC between children and adults and between patients with underlying inherited versus non inherited liver disease respectively. We included all 175 children who underwent LT for HCC and were enrolled in ELTR between 1985 and 2012; of these, 38 had an associated inherited liver disease. Adult HCC patients with (n=79) and without (n=316, matched by age, sex and LT date) inherited liver disease served as an adult comparison population. We used multivariable piecewise Cox regression models with shared frailty terms (for LT center) to compare patient and graft survival between the different HCC groups. Survival analyses demonstrated a superior long-term survival of children with inherited liver disease when compared to children with HCC without inherited liver disease (HR:0.29; 95%CI:0.10-0.90; p=0.03) and adults with HCC with inherited liver disease (HR:0.27; 95%CI:0.06-1.25;p=0.09). There was no survival difference between adults with and without inherited disease (HR:1.05; 95%CI:0.66-1.66; p=0.84).
CONCLUSION:
The potential survival advantage of children with an HCC based on inherited disease should be acknowledged when considering transplantation and prioritization for these patients. Further prospective studies accounting for tumor size and extension at LT are necessary to fully interpret our findings.