Liver transplantation for adenomatosis: European experience

• DOI: 10.1002/lt.24417
• Publication Date: 2016-02-26T13:21:08Z
• Authors: Laurence Chiche; Anaelle David; René Adam; M. Martin Oliverius; Jürgen Klempnauer; Eric Vibert; Michele Colledan; Jan Lerut; V. Vincenzo Mazza...; Stefano Di‐sandro; Christophe Laurent; Vincenzo Scuderi; Bertrand Suc; Roberto Troisi; Phillipe Bachelier; Jerôme Dumortier; Jean Gugenheim; Jean‐Yves Mabrut; Ignacio Gonzalez‐...; François‐René Pruvot; Yves Patrice Le‐T...; Francis Navarro; Jorge Ortiz‐de‐Ur...; Ephrem Salamé; Marco Spada; Paulette Bioulac‐...
• Keywords: Adult; Male; Carcinoma, Hepatocellular; Clinical Decision-Making; [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery; Glycogen Storage Disease Type I; Of the literature; Congenital absence; Adenoma, Liver Cell; Cohort Studies; Specialist hepatobiliary unit; 03 medical and health sciences; Rare Diseases; 0302 clinical medicine; Focal nodular hyperplasia; Humans; Registries; Retrospective Studies; Glycogen storage disease; Carcinoma; Liver Neoplasms; [SDV.MHEP.HEG]Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology; Adenoma, Liver Cell; Adult; Carcinoma, Hepatocellular; Clinical Decision-Making; Cohort Studies; Europe; Female; Glycogen Storage Disease Type I; Humans; Liver Neoplasms; Liver Transplantation; Male; Rare Diseases; Registries; Retrospective Studies; Treatment Outcome; Hepatocellular adenoma; Management; Liver Transplantation; 3. Good health; glycogen-storage-disease; focal nodular hyperplasia; specialist hepatobilirary unit; of-the-literature; hepatocellular adenoma; hepatic adenomatosis; portal-vein; congenital absence; management; carcinoma; Europe; Treatment Outcome; Portal vein; Female; Hepatic adenomas

The aim of this study was to collect data from patients who underwent liver transplantation (LT) for adenomatosis; to analyze the symptoms, the characteristics of the disease, and the recipient outcomes; and to better define the role of LT in this rare indication. This retrospective multicenter study, based on data from the European Liver Transplant Registry, encompassed patients who underwent LT for adenomatosis between January 1, 1986, and July 15, 2013, in Europe. Patients with glycogen storage disease (GSD) type IA were not excluded. This study included 49 patients. Sixteen patients had GSD, and 7 had liver vascular abnormalities. The main indications for transplantation were either a suspicion of hepatocellular carcinoma (HCC; 15 patients) or a histologically proven HCC (16 patients), but only 17 had actual malignant transformation (MT) of adenomas. GSD status was similar for the 2 groups, except for age and the presence of HCC on explants (P = 0.030). Three patients with HCC on explant developed recurrence after transplantation. We obtained and studied the pathomolecular characteristics for 23 patients. In conclusion, LT should remain an extremely rare treatment for adenomatosis. Indications for transplantation primarily concern the MT of adenomas. The decision should rely on morphological data and histological evidence of MT. Additional indications should be discussed on a case‐by‐case basis. In this report, we propose a simplified approach to this decision‐making process.