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Where do we stand with hepatoblastoma?

A review

  1. J. Marco Schnater M.D.1,2,3,
  2. S. Eleonore Köhler Ph.D.2,
  3. Wouter H. Lamers M.D.3,
  4. Dietrich von Schweinitz M.D.2,
  5. Daniël C. Aronson M.D.1,†,*

Article first published online: 16 JUL 2003

DOI: 10.1002/cncr.11585

Issue

Cancer

Cancer

Volume 98, Issue 4, pages 668–678, 15 August 2003

Additional Information

How to Cite

Schnater, J. M., Köhler, S. E., Lamers, W. H., von Schweinitz, D. and Aronson, D. C. (2003), Where do we stand with hepatoblastoma?. Cancer, 98: 668–678. doi: 10.1002/cncr.11585

Author Information

  1. 1

    Pediatric Surgical Center of Amsterdam, Emma Children's Hospital AMC, Academic Medical Center, Amsterdam, The Netherlands

  2. 2

    Department of Pediatric Surgery, University Children's Hospital, Basel, Switzerland

  3. 3

    AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands

  1. †

    Fax: (011) 31 20566-9287

*Pediatric Surgical Center of Amsterdam, Emma Children's Hospital AMC, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands

Publication History

  1. Issue published online: 1 AUG 2003
  2. Article first published online: 16 JUL 2003
  3. Manuscript Accepted: 19 MAY 2003
  4. Manuscript Revised: 27 APR 2003
  5. Manuscript Received: 31 DEC 2002

Funded by

  • TerMeulen Fund
  • Royal Netherlands Academy of Arts and Sciences

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Keywords:

  • pediatric tumors;
  • phenotypic characteristics;
  • staging systems;
  • treatment;
  • prognosis

Abstract

Hepatoblastoma (HB) is the most common pediatric liver malignancy, comprising approximately 1% of all pediatric cancers. The disparate clinical staging systems and histologic classifications that were developed during the last decades, nevertheless, reflect the remaining difficulties and uncertainties in characterizing HB. Furthermore, the combination of surgery and (neo)adjuvant chemotherapy has improved patient outcomes dramatically. A poor prognosis is associated with large tumor size, multifocality, extrahepatic disease, and metastatic spread. The exact etiology of HB remains unknown, but the cytogenetic alterations, phenotypic features, and biologic aspects that accompany this neoplasm yield more and more insight into its pathogenesis. New cell-biologic and molecular-biologic insights may lead to the development of new treatment modalities, especially for patients with a bad prognosis. This review summarizes the different aspects of this intriguing tumor and discusses the current status of research and treatment for patients with HB. Cancer 2003;98:668–78. © 2003 American Cancer Society.

DOI 10.1002/cncr.11585

View Full Article (HTML) Get PDF (195K)