Pathogenesis and clinical management of hereditary non-polyposis colorectal cancer
- 1 November 2002
- journal article
- review article
- Published by Oxford University Press (OUP) in British Journal of Surgery
- Vol. 89 (11), 1357-1369
- https://doi.org/10.1046/j.1365-2168.2002.02290.x
Abstract
Background Hereditary non-polyposis colorectal cancer (HNPCC) is an inherited genetic condition associated with microsatellite instability; it accounts for around 5 per cent of all cases of colorectal cancer. This review examines recent data on management strategies for this condition. Methods A Medline-based literature search was performed using the keywords ‘HNPCC’ and ‘microsatellite instability’. Additional original papers were obtained from citations in articles identified by the initial search. Results and conclusion The Amsterdam criteria identify patients in whom the presence of an inherited mutation should be investigated. Those with a mutation should be offered counselling and screening. The role of prophylactic surgery has been superseded by regular colonoscopy, which dramatically reduces the risk of colorectal cancer. Screening for extracolonic malignancy is also advocated, but the benefits are uncertain. Chemoprevention may be of value in lowering the incidence of bowel cancer in affected patients, but further studies are required.Keywords
This publication has 100 references indexed in Scilit:
- Association of tumour site and sex with survival benefit from adjuvant chemotherapy in colorectal cancerThe Lancet, 2000
- Genetic pathways in colorectal and other cancersEuropean Journal Of Cancer, 1999
- Hereditary cancer: Two hits revisitedZeitschrift für Krebsforschung und Klinische Onkologie, 1996
- Life‐time risk of different cancers in hereditary non‐polyposis colorectal cancer (hnpcc) syndromeInternational Journal of Cancer, 1995
- Genetic linkage in Muir-Torre syndrome to the same chromosomal region as cancer family syndromeEuropean Journal Of Cancer, 1994
- Hereditary nonpoiyposis colorectal cancer (lynch syndrome II): Diploid malignancies with prolonged survivalCancer, 1992
- The International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer (ICG-HNPCC)Diseases of the Colon & Rectum, 1991
- Clinical features of colorectal carcinoma in cancer family syndromeDiseases of the Colon & Rectum, 1986
- Effect of Added Dietary Calcium on Colonic Epithelial-Cell Proliferation in Subjects at High Risk for Familial Colonic CancerNew England Journal of Medicine, 1985
- Prolonged survival as a component of hereditary breast and nonpolyposis colon cancerMedical Hypotheses, 1981