Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Hepatoblastoma

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_2662-2

Definition

Hepatoblastoma is a childhood malignant embryonal liver tumor consisting of immature epithelial cells with or without additional mesenchymal component.

Characteristics

Hepatoblastomas are the most frequent malignant liver tumors of childhood. Their annual incidence is 0.5–1 cases per million children under 15 years of age in the Western countries. The affected children are most frequently between 6 months and 3 years old. Hepatoblastoma has also been detected in utero by prenatal ultrasound examination. Since liver tumors lack early clinical symptoms, hepatoblastoma patients often present with locally extended tumors at diagnosis. However, distant metastases usually occur very late in the disease progression. The patients frequently have highly elevated α-fetoprotein levels. In these cases this oncofetal antigen can be useful as a sensitive diagnostic marker and also as a marker for the monitoring of treatment response.

Pathological Classification

Hepatoblastomas always...

Keywords

Hepatocyte Growth Factor Familial Adenomatous Polyposis Adenomatous Polyposis Coli Embryonal Liver Liver Progenitor Cell 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Brown J, Perilongo G, Shafford E et al (2000) Pretreatment prognostic factors for children with hepatoblastoma – results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1. Eur J Cancer 36:1418–1425CrossRefPubMedGoogle Scholar
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  3. Von Schweinitz D, Hecker H, Schmidt-von-Arndt G et al (1997a) Prognostic factors and staging systems in childhood hepatoblastoma. Int J Cancer 74:593–599CrossRefGoogle Scholar
  4. Von Schweinitz D, Byrd DJ, Hecker H et al (1997b) Efficiency and toxicity of ifosfamide, cisplatin and doxorubicin in the treatment of childhood hepatoblastoma. Study Committee of the Cooperative Paediatric Liver Tumour Study HB89 of the German Society for Paediatric Oncology and Haematology. Eur J Cancer 33:1243–1249CrossRefGoogle Scholar
  5. Weinberg AG, Finegold MJ (1983) Primary hepatic tumors of childhood. Hum Pathol 14:512–537CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Institut für Neuropathologie, KinderchirurgieUniversitätskliniken BonnBonnGermany
  2. 2.Klinikum der Universität München, Kinderchirurgische Klinik im Dr. von Haunerschen KinderspitalMünchenGermany