Familial Colorectal Cancer Type X

The Other Half of Hereditary Nonpolyposis Colon Cancer Syndrome

Research output: Contribution to journalArticle

54 Citations (Scopus)

Abstract

Establishing the Amsterdam criteria, based on pedigrees, was essential for defining hereditary nonpolyposis colorectal cancer (HNPCC) syndrome in such a way that the underlying genetic cause could be identified. It is now known that about half of families that fulfill the original Amsterdam criteria have a hereditary DNA mismatch repair (MMR) gene mutation. These families may be said to have Lynch syndrome. The other half of families with HNPCC has no evidence of DNA MMR deficiency, and studies show that these families are different from families with Lynch syndrome. Familial colorectal cancer type X is the name used to refer to the "other half of HNPCC".

Original languageEnglish (US)
Pages (from-to)637-645
Number of pages9
JournalSurgical Oncology Clinics of North America
Volume18
Issue number4
DOIs
StatePublished - Oct 2009

Fingerprint

Hereditary Nonpolyposis Colorectal Neoplasms
Colorectal Neoplasms
DNA Mismatch Repair
DNA Repair-Deficiency Disorders
Pedigree
Names
Mutation
Genes

Keywords

  • Cancer
  • Colorectal
  • Familial
  • Hereditary
  • HNPCC
  • Nonpolyposis

ASJC Scopus subject areas

  • Oncology
  • Surgery

Cite this

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