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Review article: The Lynch syndrome (hereditary nonpolyposis colorectal cancer).
Aliment Pharmacol Ther 2007; 26 Suppl 2:113-26AP

Abstract

BACKGROUND

The most common inherited colorectal cancer syndrome is the Lynch syndrome (HNPCC) which is characterized by the development of colorectal, endometrial, and other cancers and the presence of microsatellite instability (MSI) in tumours. The syndrome is due to a mutation in one of the mismatch repair (MMR) genes: MSH2, MLH1, MSH6 and PMS2.

AIMS

To evaluate the clinical features of the Lynch syndrome and to assess the tools that are available for the identification of families with this syndrome.

METHODS

A systematic literature search using PubMed and reference lists of retrieved articles was performed.

RESULTS

The review provides an update of the clinical phenotype of the Lynch syndrome. Until recently, the Amsterdam criteria were the most important tool for the identification of Lynch syndrome. Nowadays, the Bethesda guidelines are more widely used. These guidelines describe all clinical conditions in which a search for MSI indicated. Both MSI-analysis as well as immunohistochemical analysis of the MMR-proteins are appropriate to identify patients with a high probability of carrying a MMR-gene mutation.

CONCLUSION

All specialists that are involved in the treatment of cancer patients should know the Bethesda criteria in order to identify all families suspected of the Lynch syndrome.

Authors+Show Affiliations

Department of Gastroenterology & Medical Oncology, Leiden University Medical Centre, Leiden, The Netherlands. hfavasen@stoet.nl

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18081655

Citation

Vasen, H F A.. "Review Article: the Lynch Syndrome (hereditary Nonpolyposis Colorectal Cancer)." Alimentary Pharmacology & Therapeutics, vol. 26 Suppl 2, 2007, pp. 113-26.
Vasen HF. Review article: The Lynch syndrome (hereditary nonpolyposis colorectal cancer). Aliment Pharmacol Ther. 2007;26 Suppl 2:113-26.
Vasen, H. F. (2007). Review article: The Lynch syndrome (hereditary nonpolyposis colorectal cancer). Alimentary Pharmacology & Therapeutics, 26 Suppl 2, pp. 113-26. doi:10.1111/j.1365-2036.2007.03479.x.
Vasen HF. Review Article: the Lynch Syndrome (hereditary Nonpolyposis Colorectal Cancer). Aliment Pharmacol Ther. 2007;26 Suppl 2:113-26. PubMed PMID: 18081655.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Review article: The Lynch syndrome (hereditary nonpolyposis colorectal cancer). A1 - Vasen,H F A, PY - 2008/1/8/pubmed PY - 2008/4/12/medline PY - 2008/1/8/entrez SP - 113 EP - 26 JF - Alimentary pharmacology & therapeutics JO - Aliment. Pharmacol. Ther. VL - 26 Suppl 2 N2 - BACKGROUND: The most common inherited colorectal cancer syndrome is the Lynch syndrome (HNPCC) which is characterized by the development of colorectal, endometrial, and other cancers and the presence of microsatellite instability (MSI) in tumours. The syndrome is due to a mutation in one of the mismatch repair (MMR) genes: MSH2, MLH1, MSH6 and PMS2. AIMS: To evaluate the clinical features of the Lynch syndrome and to assess the tools that are available for the identification of families with this syndrome. METHODS: A systematic literature search using PubMed and reference lists of retrieved articles was performed. RESULTS: The review provides an update of the clinical phenotype of the Lynch syndrome. Until recently, the Amsterdam criteria were the most important tool for the identification of Lynch syndrome. Nowadays, the Bethesda guidelines are more widely used. These guidelines describe all clinical conditions in which a search for MSI indicated. Both MSI-analysis as well as immunohistochemical analysis of the MMR-proteins are appropriate to identify patients with a high probability of carrying a MMR-gene mutation. CONCLUSION: All specialists that are involved in the treatment of cancer patients should know the Bethesda criteria in order to identify all families suspected of the Lynch syndrome. SN - 1365-2036 UR - https://www.unboundmedicine.com/medline/citation/18081655/Review_article:_The_Lynch_syndrome__hereditary_nonpolyposis_colorectal_cancer__ L2 - https://doi.org/10.1111/j.1365-2036.2007.03479.x DB - PRIME DP - Unbound Medicine ER -