Paediatric hepatocellular adenomas: Lessons from a systematic review of relevant literature.

Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterise paediatric HCAs and determine their course, complications, and management. Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive review of the published literature. A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) and at a mean age of 11.5 (range 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and MODY3 (maturity-onset diabetes of the young type 3). Each of these diseases leads to a well-defined HCA molecular pattern. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in children with portosystemic shunts and in β-catenin-mutated HCAs, while haemorrhages were more frequent in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat adenomatosis, as well as any underlying diseases. Progress in understanding genetic and/or malformative contributions, which appear to be significant in paediatric HCAs, have provided insights into tumour pathogenesis and will further guide patient surveillance and management.

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© 2024 The Authors.

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IS is supported by Post-Doctoral and Research Grants from Fond National pour la Recherche Scientifique (FNRS), Fondation Contre le Cancer (FCC), Fondation Saint-Luc contre le cancer and Fondation Salus Sanguinis. XS is supported by Grant Fond de Recherche Clinique (FRC) from the Cliniques universitaires Saint-Luc. XS has received consulting fees (outside the field of hepatocellular adenomas) from Mirum and Ipsen. AP is supported by research Grants (outside the field of hepatocellular adenomas) from Fondation Nuovo-Soldati, Fond national de la recherche scientifique (Télévie Grant number 7.4514.22) and Salus Sanguinis. JCN is supported by INSERM, Institut National du Cancer (INCa) PRTK 2014 MUTHEC, Ligue National contre le Cancer (Equipe labellisée), ANRS CSS7 AAP 2018-1 CITHEC, Association Française pour l’Étude du Foie (AFEF) 2022 projet radio-moléculaire, Agence Nationale De La Recherche (ANR) 2022 SYSTHEC, Agence nationale de recherches sur le sida et les hépatites virales (ANRS) 2023 CSS13 HBV-LIRAGE ECTZ232901, SIRIC CAncer Research in multiple dimensions to accelerate PrEcision Medicine (CARPEM) INCa-DGOS-Inserm-12561. JCN has received research grants (outside the field of hepatocellular adenomas) from Bayer and Ipsen. RT, CDM and RR have no disclosures. All authors have approved the final manuscript. Please refer to the accompanying ICMJE disclosure forms for further details.