title: [Hepatic tumors in childhood: experience on 245 tumors and review of literature].
abstract: This review on the pathology of hepatic tumors in childhood, from a personal series of 245 tumors, focuses on incidence, management, description of frequent tumors such as hepatoblastoma, fibrolamellar carcinoma, and undifferentiated sarcoma for malignant tumors, focal nodular hyperplasia, hepatocellular adenoma, and mesenchymal hamartoma for benign tumors. Malignant and benign entities of recent description, including the following: crowded, small cell undifferentiated and cholangioblastic variants of hepatoblastomas, mesenchymal hamartoma miming hepatoblastoma, liver adenoma and adenomatosis in diabete MODY3 families, gastrointestinal stromal tumor with liver metastasis associated to Carney triad, macronodules in non-cirrhotic portal fibrosis are reviewed. For each entity, the clinical presentation, the diagnostic criteria and the differential diagnosis are described. The role of immunohistochemistry and molecular biology in the diagnosis and identification of new molecular mechanisms triggered by oncogenic activation with new prognostic markers, and therapeutic targets is emphasized.
authors.affiliation: Service d'Anatomie et de Cytologie Pathologiques, Faculté de Médecine Paris XI et Hôpital de Bicêtre, 78, rue du Général Leclerc, 94270 Le Kremlin Bicêtre, France. monique.fabre@bct.ap-hop-paris.fr
authors.forename: Monique
authors.initials: M
authors.lastname: Fabre
authors.forename: Funda
authors.initials: F
authors.lastname: Yilmaz
authors.forename: Marie Annick
authors.initials: MA
authors.lastname: Buendia
mesh_terms: D002648:Child; D018197:Hepatoblastoma / Q000145:classification / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000145:classification / Q000453:epidemiology / Q000473:pathology* / Q000556:secondary / Q000628:therapy