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Extracolonic Tumors

  • Benedito Mauro RossiEmail author
  • Fabio de Oliveira Ferreira
Chapter
Part of the M.D. Anderson Solid Tumor Oncology Series book series (MDA, volume 5)

Abstract

Lynch Syndrome (LS), or Hereditary non-polyposis colorectal cancer (HNPCC), is caused by inherited germline mutations in mismatch repair (MMR) genes. It is one of the commonest forms of inherited predisposition to colorectal cancer (CRC), accounting for 2%–5% of all CRC. LS is characterized by early age of onset, with a tendency for multiplicity of tumors, and an increased risk for extracolonic tumors at particular sites. In this chapter, the LS extracolonic tumors characteristics are presented, including tumor spectrum and lifetime risk of cancer. Some specific types of cancer, such as endometrium, urinary tract, small bowel, brain, stomach, hepatobiliary tract, pancreas, skin and breast are discussed in detail.

Keywords

Lynch syndrome Hereditary tumors Colorectal cancer Extracolonic cancer Tumor spectrum Lifetime risk 

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© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Benedito Mauro Rossi
    • 1
    Email author
  • Fabio de Oliveira Ferreira
  1. 1.Hereditary Cancer RegistryHospital de Cancer de BarretosSão PauloBrazil

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