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Hereditary non-polyposis colorectal cancer or Lynch syndrome: the gynaecological perspective.
Curr Opin Obstet Gynecol 2009; 21(1):31-8CO

Abstract

PURPOSE OF REVIEW

Hereditary non-polyposis colorectal cancer (HNPCC) or Lynch syndrome is characterized by a number of other cancers including colorectal, endometrial and ovarian cancer. This review covers the gynaecological aspects of managing women with HNPCC: diagnostic criteria, molecular tests for diagnosis, cancer risks and different strategies for surveillance and prevention.

RECENT FINDINGS

Studies correcting for ascertainment bias found slightly lower penetrance estimates than those obtained from high-risk families. HNPCC linked ovarian cancer presents at an earlier age and stage and has similar survival rates as sporadic cancer. In endometrial tumours, microsatellite instability or immunohistochemistry has limited effectiveness in selecting individuals for genetic testing, due to molecular differences. Population-based data indicate that a significant proportion of mismatch repair gene carriers would be missed by current clinical criteria. Effective risk prediction models complement clinical risk assessment. Effectiveness of screening is unproven and prophylactic surgery is the best preventive option for women who have completed their families. Multimodal screening protocols from the age of 30-35 years are being evaluated.

SUMMARY

Risk of endometrial cancer in women with Lynch syndrome is as high as the risk of colorectal cancer. Further research is needed to identify the appropriate strategy for clinical risk assessment and optimize screening. A multidisciplinary approach is necessary to manage these women.

Authors+Show Affiliations

Department of Gynaecological Oncology, Institute for Women's Health, University College London, London, UK. r.manchanda@ucl.ac.ukNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

19125001

Citation

Manchanda, Ranjit, et al. "Hereditary Non-polyposis Colorectal Cancer or Lynch Syndrome: the Gynaecological Perspective." Current Opinion in Obstetrics & Gynecology, vol. 21, no. 1, 2009, pp. 31-8.
Manchanda R, Menon U, Michaelson-Cohen R, et al. Hereditary non-polyposis colorectal cancer or Lynch syndrome: the gynaecological perspective. Curr Opin Obstet Gynecol. 2009;21(1):31-8.
Manchanda, R., Menon, U., Michaelson-Cohen, R., Beller, U., & Jacobs, I. (2009). Hereditary non-polyposis colorectal cancer or Lynch syndrome: the gynaecological perspective. Current Opinion in Obstetrics & Gynecology, 21(1), pp. 31-8. doi:10.1097/GCO.0b013e32831c844d.
Manchanda R, et al. Hereditary Non-polyposis Colorectal Cancer or Lynch Syndrome: the Gynaecological Perspective. Curr Opin Obstet Gynecol. 2009;21(1):31-8. PubMed PMID: 19125001.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hereditary non-polyposis colorectal cancer or Lynch syndrome: the gynaecological perspective. AU - Manchanda,Ranjit, AU - Menon,Usha, AU - Michaelson-Cohen,Rachel, AU - Beller,Uziel, AU - Jacobs,Ian, PY - 2009/1/7/entrez PY - 2009/1/7/pubmed PY - 2009/6/16/medline SP - 31 EP - 8 JF - Current opinion in obstetrics & gynecology JO - Curr. Opin. Obstet. Gynecol. VL - 21 IS - 1 N2 - PURPOSE OF REVIEW: Hereditary non-polyposis colorectal cancer (HNPCC) or Lynch syndrome is characterized by a number of other cancers including colorectal, endometrial and ovarian cancer. This review covers the gynaecological aspects of managing women with HNPCC: diagnostic criteria, molecular tests for diagnosis, cancer risks and different strategies for surveillance and prevention. RECENT FINDINGS: Studies correcting for ascertainment bias found slightly lower penetrance estimates than those obtained from high-risk families. HNPCC linked ovarian cancer presents at an earlier age and stage and has similar survival rates as sporadic cancer. In endometrial tumours, microsatellite instability or immunohistochemistry has limited effectiveness in selecting individuals for genetic testing, due to molecular differences. Population-based data indicate that a significant proportion of mismatch repair gene carriers would be missed by current clinical criteria. Effective risk prediction models complement clinical risk assessment. Effectiveness of screening is unproven and prophylactic surgery is the best preventive option for women who have completed their families. Multimodal screening protocols from the age of 30-35 years are being evaluated. SUMMARY: Risk of endometrial cancer in women with Lynch syndrome is as high as the risk of colorectal cancer. Further research is needed to identify the appropriate strategy for clinical risk assessment and optimize screening. A multidisciplinary approach is necessary to manage these women. SN - 1473-656X UR - https://www.unboundmedicine.com/medline/citation/19125001/Hereditary_non_polyposis_colorectal_cancer_or_Lynch_syndrome:_the_gynaecological_perspective_ L2 - http://Insights.ovid.com/pubmed?pmid=19125001 DB - PRIME DP - Unbound Medicine ER -