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Orpha number ORPHA449
Synonym(s) -
Prevalence Unknown
Inheritance Not applicable
Age of onset Childhood
  • C22.2
  • C0206624
  • D018197
  • 10062001
  • 109843000
  • 45024009


Hepatoblastoma (HB) is a rare tumour but represents the commonest primary malignant tumour of the liver in childhood. It accounts for about 1% of all childhood tumours with an annual incidence of 1.5 cases per million children younger than 15 years. The onset of HB occurs at a median age of 18-24 months. Serum alpha-fetoprotein levels are frequently elevated. Main symptoms are palpable hepatic mass and abdominal pain. Systemic symptoms are rare. HB might be associated with Beckwith-Wiedemann syndrome and familial adenomatous polyposis. Treatment of HB combines cisplatin-based chemotherapy and surgery. In Europe, HB is typically treated with preoperative chemotherapy, followed by tumour resection and a short course of postoperative chemotherapy, whereas primary surgery followed by adjuvant chemotherapy is the recommended treatment strategy in the United States.

Expert reviewer(s)

  • Pr Giorgio PERILONGO

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Detailed information

Clinical practice guidelines
  • DE (2010)
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