Chapter 5. Hereditary Brain Tumors

  1. Prof. Dr. Heike Allgayer PhD2,
  2. Prof. Dr. Helga Rehder3 and
  3. Prof. Dr. Simone Fulda4
  1. Christine Marosi

Published Online: 21 AUG 2009

DOI: 10.1002/9783527627523.ch5

Hereditary Tumors: From Genes to Clinical Consequences

Hereditary Tumors: From Genes to Clinical Consequences

How to Cite

Marosi, C. (2008) Hereditary Brain 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.ch5

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. Medizinische Universität Wien, Allgemeines Krankenhaus, Klinische Abteilung für Onkologie, Währinger Gürtel 18–20, 1090 Vienna, Austria

Publication History

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

ISBN Information

Print ISBN: 9783527320288

Online ISBN: 9783527627523



  • central nervous system;
  • glioma;
  • hereditary brain tumors;
  • NF2 mutations;
  • rhabdoid;
  • schwannoma


This chapter provides a comprehensive summary on hereditary brain tumor syndromes which are: Cowden syndrome/Lhermitte–Duclos syndrome, Li-Fraumen syndrome, Naevoid basal cell carcinoma syndrome (NBCCS)/Gorlin syndrome, Neurofibromatosis I, Neurofibromatosis II, Rhabdoid tumor predisposition syndrome, Tuberous sclerosis, Turcot syndrome, and Von Hippel–Lindau syndrome. They involve tumor suppressor genes and are inherited as autosomal dominant (AD) traits. Since most of the syndromes associated with brain tumors are also cancer family syndromes, the description is focused on the central nervous system (CNS) manifestations, and the reader is advised to also consider the Chapters 3 and 6 on family cancer syndromes and neurofibromatosis of this book.