title: Primitive malignant nonepithelial hepatic tumors in children.
abstract: Primary intrahepatic malignant nonepithelial tumors are very rare in children and account for 2% of all malignant mesenchymatous tumors under the age of 15 years. Clinical presentation, radiologic features, and histologic types are not unequivocal. The predominant role of surgery takes place either initially in small localized tumors or later, after initial reductive chemotherapy. In all cases, complete resection is the necessary but not sufficient condition for cure. Additional radiotherapy seems ineffective. High-dose chemotherapy and/or liver transplantation can be proposed for resistant cases. The disease-free survival rate is 37% at 2 years for the whole series.
authors.affiliation: Service d'Onco-Hematologie, Centre Hospitalier Régional, Strasbourg, France.
authors.forename: A
authors.initials: A
authors.lastname: Babin-Boilletot
authors.forename: F
authors.initials: F
authors.lastname: Flamant
authors.forename: M J
authors.initials: MJ
authors.lastname: Terrier-Lacombe
authors.forename: H B
authors.initials: HB
authors.lastname: Marsden
authors.forename: A
authors.initials: A
authors.lastname: van Unnik
authors.forename: F
authors.initials: F
authors.lastname: Deméocq
authors.forename: J M
authors.initials: JM
authors.lastname: Zucker
authors.forename: P A
authors.initials: PA
authors.lastname: Voûte
authors.forename: J
authors.initials: J
authors.lastname: Otten
authors.forename: C
authors.initials: C
authors.lastname: Behar
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D008297:Male; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D011379:Prognosis; D012074:Remission Induction; D012086:Reoperation; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000473:pathology* / Q000556:secondary / Q000601:surgery / Q000628:therapy*; D012509:Sarcoma / Q000188:drug therapy / Q000473:pathology* / Q000556:secondary / Q000601:surgery / Q000628:therapy*