Chapter 21. Liver Tumors

  1. Prof. Dr. Heike Allgayer PhD2,
  2. Prof. Dr. Helga Rehder3 and
  3. Prof. Dr. Simone Fulda4
  1. Sabine J. Presser

Published Online: 21 AUG 2009

DOI: 10.1002/9783527627523.ch21

Hereditary Tumors: From Genes to Clinical Consequences

Hereditary Tumors: From Genes to Clinical Consequences

How to Cite

Presser, S. J. (2008) Liver Tumors, in Hereditary Tumors: From Genes to Clinical Consequences (eds H. Allgayer, H. Rehder and S. Fulda), Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim, Germany. doi: 10.1002/9783527627523.ch21

Editor Information

  1. 2

    University of Heidelberg and DKFZ (German Cancer Research Center) Heidelberg, Medical Faculty Mannheim, Chair of Experimental Surgery, Theodor-Kutzer-Ufer 1–3, 68167 Mannheim, Germany

  2. 3

    Medical University Vienna, Department of Medical Genetics, Währinger Strasse 10, 1090 Wien, Austria

  3. 4

    Ulm University Children's Hospital, Eythstrasse 24, 89075 Ulm, Germany

Author Information

  1. Charité Campus Virchow-Klinikum, Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Augustenburger Platz 1, 13353 Berlin, Germany

Publication History

  1. Published Online: 21 AUG 2009
  2. Published Print: 17 DEC 2008

ISBN Information

Print ISBN: 9783527320288

Online ISBN: 9783527627523



  • Alagille syndrome;
  • Beckwith–Wiedemann syndrome;
  • cystic fibrosis;
  • hemochromatosis;
  • hepatoblastoma;
  • hereditary tyrosinemia;
  • Wilson's disease


In contrast to other tumor entities, such as colon and mamma adenocarcinomas, hereditary aspects of malignant liver tumors have only been intensely studied within recent decades. Exogenous factors, such as viral hepatitis and food carcinogens, are traditionally regarded as hallmarks of liver carcinogenesis. However, the individual risk to develop liver cancer due to these factors also depends on a number of hereditary factors, such as susceptibility for viral infections and genetic variations in metabolizing liver enzymes, such as cytochrome P450 cyclooxogenases. Whereas hereditary aspects of carcinogen activation and detoxification are reviewed below, we would like to refer the interested reader to specific immunological literature with respect to the influence of host genetics on viral infections.

In addition, many inherited metabolic diseases significantly enhance the risk of liver cancer, and so are described in detail in this respect. They often follow a chronic progressive course and are linked to liver fibrosis. The molecular and cellular mechanisms responsible for malignant transformation of hepatocytes and biliary epithelial cells are not well understood. However, it appears that exogeneous factors also influence the cancer risk of inherited liver diseases.

Finally, a number of oncogenes and tumor suppressor genes are shown to be involved in the development and progression of liver cancer. Therefore, a brief description of the involved genes is included at the end of this chapter.