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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