6. Hepatoblastoma

  1. Ross Pinkerton MB, BCh, BaO, MD Executive Director, Division of Oncology2,
  2. Ananth Shankar MD, FRCPCH Consultant in Paediatric and Adolescent Oncology3 and
  3. Katherine K. Matthay BA, MD Mildred V. Strouss Professor of Translational Oncology, Director, Pediatric Hematology-Oncology4
  1. Ross Pinkerton

Published Online: 8 MAY 2013

DOI: 10.1002/9781118625309.ch6

Evidence-Based Pediatric Oncology

Evidence-Based Pediatric Oncology

How to Cite

Pinkerton, R. (2013) Hepatoblastoma, in Evidence-Based Pediatric Oncology (eds R. Pinkerton, A. Shankar and K. K. Matthay), John Wiley & Sons, Ltd, Oxford. doi: 10.1002/9781118625309.ch6

Editor Information

  1. 2

    Royal Children's Hospital, Children's Health Queensland, Brisbane, QLD, Australia

  2. 3

    University College London Hospitals NHS Foundation Trust London, UK

  3. 4

    Department of Pediatrics, UCSF School of Medicine and, UCSF Benioff Children's Hospital, San Francisco, CA, USA

Author Information

  1. Royal Children's Hospital, Brisbane, QLD, Australia

Publication History

  1. Published Online: 8 MAY 2013
  2. Published Print: 20 MAY 2013

ISBN Information

Print ISBN: 9780470659649

Online ISBN: 9781118625309

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Keywords:

  • chemotherapy;
  • childhood cancers;
  • hepatoblastoma (HB);
  • liver tumors

Summary

Liver tumors can be primary or secondary, benign or malignant. The most common primary liver cancer is hepatoblastoma (HB) and most tumors secrete a-fetoprotein (AFP). Childhood cancers are rare and therefore hepatoblastoma is exceedingly rare, affecting about one in a million children. Despite its rarity, international collaboration and successive international clinical trials have made it one of the success stories of the last decades, improving the cure rate from 30% to the vast majority of children. The two-thirds of children who have standard-risk or good prognostic disease are now curable, and over 80% are disease free at 3 years and beyond, with a combination of chemotherapy and surgery. In the remaining third with high-rise disease, clearance in the liver may require liver transplantation and clearance in the lungs requires dose-intensive chemotherapy. However, with these modalities, over 70% are disease free at 2 years and beyond.