title: [Paediatric malignant liver tumours].
abstract: Tumours and pseudotumours of the liver are a heterogeneous group of neoplasm including 60% of malignant tumours. Malignant liver tumours account for less than 2% of the lesions in children and vary considerably in incidence throughout the paediatric age range, with hepatoblastoma, rhabdoid tumour of the liver, hemangioendothelioma, biliary tract rhabdomysosarcoma and mesenchymal hamartoma in the first two years of life and hepatocellular carcinoma, focal nodular hyperplasia, and undifferentiated sarcoma in older children and adolescents. Treatment of malignant epithelial tumours is based on the surgical resection of the tumour associated with pre- and postoperative chemotherapy including cisplatinum. Modalities of the treatment are adapted to risk factors. Survival rates at three years are over 80% for localised hepatoblastoma whereas they are less than 30% in hepatocellular carcinomas. The role of targeted therapies still has to be defined.
authors.affiliation: Institut Gustave-Roussy, département d'oncologie de l'enfant et de l'adolescent, 114, rue Édouard-Vaillant, 94805 Villejuif, France. Laurence.brugieres@igr.fr
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
authors.forename: Sophie
authors.initials: S
authors.lastname: Branchereau
authors.forename: Véronique
authors.initials: V
authors.lastname: Laithier
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001661:Biliary Tract Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D006390:Hemangioendothelioma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D018197:Hepatoblastoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D018335:Rhabdoid Tumor / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D015996:Survival Rate