Familial Colorectal Cancer Type X: The Other Half of Hereditary Nonpolyposis Colon Cancer Syndrome

Noralane M. Lindor

Research output: Contribution to journalReview articlepeer-review

60 Scopus citations

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

Keywords

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

ASJC Scopus subject areas

  • Surgery
  • Oncology

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