Hepatic tumors in childhood: experience on 245 tumors and review of literature

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 hamortoma 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 ore 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.