Chapter 27. Sarcomas and Bone Tumors in Adulthood

  1. Prof. Dr. Heike Allgayer PhD2,
  2. Prof. Dr. Helga Rehder3 and
  3. Prof. Dr. Simone Fulda4
  1. Eva Wardelmann

Published Online: 21 AUG 2009

DOI: 10.1002/9783527627523.ch27

Hereditary Tumors: From Genes to Clinical Consequences

Hereditary Tumors: From Genes to Clinical Consequences

How to Cite

Wardelmann, E. (2008) Sarcomas and Bone Tumors in Adulthood, 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.ch27

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. Universitätsklinikum Bonn, Institut für Pathologie, Sigmund-Freud-Strasse 25, 53127 Bonn, Germany

Publication History

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

ISBN Information

Print ISBN: 9783527320288

Online ISBN: 9783527627523



  • enchondromatosis;
  • familial adenomatous polyposis;
  • Mendelian;
  • multiple osteochondromas;
  • neoplasm;
  • osteochondromas


As for sporadic tumors, recent studies have improved our understanding of the genetic background of hereditary tumors of soft tissue and bone. A large number of syndromes associated with neoplasms is well-known and is becoming more precisely characterized. The knowledge of the Mendelian inheritance of different diseases is the prerequisite for counseling of patients and relatives to prevent life-threatening stages of disease by early recognition and appropriate treatment. Furthermore, the knowledge of which pathways might be involved in the development of specific soft tissue and bone tumors will probably help to also better understand sporadic tumors.

The minority of bone and soft tissue tumors is due to inherited susceptibility, often associated with syndromes. In the following chapter, several syndromes with known or highly suspected underlying genetic causes are described, whereas rare groups with a very low incidence are not. As such, the list is not complete and preferentially comprises the most common syndromes. The overlap with other chapters concerning familial syndromes is not avoidable, but this chapter focuses on the tumor manifestations in soft tissue and bone.