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Monday, February 17, 2014

What is Lynch Syndrome?

Lynch Syndrome is a hereditary condition that places people at an increased risk of developing certain types of cancers. People with Lynch Syndrome have a problem with DNA replication which creates a gene mismatch. This mismatch predisposes individuals to developing cancers of the colon, endometrium, stomach, ovary, liver, pancreas, small bowel, urinary tract, brain and the sweat glands of the skin. These cancers often occur earlier in life than cancer in the general population and generally before the age of 50. Once Lynch Syndrome is identified in an individual, family members should be screened to determine if they also have Lynch Syndrome. If a parent has Lynch Syndrome, their children have a 50% chance of also inheriting the condition.

Identifying Lynch Syndrome early in life allows individuals to follow surveillance plans for cancer prevention. Careful follow up for individuals and families lowers the risk of dying from Lynch Syndrome related cancers.

The chart below shows the lifetime risk of certain cancers for people with Lynch Syndrome as compared with people without Lynch Syndrome in the general population.


 
Cancer Risk of Individuals with Lynch Syndrome without Screening & Surveillance vs. Non Lynch Risk
CANCER
LYNCH RISK
NON LYNCH RISK
Colon
     50-82%
5-6%
Endometrium
25-60%
2-3%
Stomach
                  6-13%
Less than 1%
Ovary
                  4-12%
1-2%
Hepatobiliary
                  1.5-4%
Less than 1%
Small Bowel
                  3-6%
Less than1%
Urinary
                  1-4%
Less than1%
Brain
                  1-3%
Less than1%
Skin (sebaceous)
                  1-9%
Less than1%
Pancreas
                  1-6%
Less than1%
Data taken from Dinh et al. (2011)

To watch a video about Lynch Syndrome by Heather Hampel, a genetic counselor at The Ohio State University Comprehensive Cancer Cancer. Click the photo below if you have Adobe Flash Player installed or if not click the link below the photo.




OR


References

Dinh, A. D., Rosner, B.I., Atwood, J. C., Boland, C. R., Syngal, S., Vasen, H. F., … Burt, R. W. (2011). Health benefits and cost-effectiveness of primary genetic screening for Lynch Syndrome in the general population. Cancer Prevention and Research, 4, 9–22.  Retrieved from http://cancerpreventionresearch.aacrjournals.org/content/4/1/9.full. doi:10.1158/1940-6207.CAPR-10-0262



Lynch, H. T., Jascur, T., Lanspa, S. C. & Boland. R. (2010). Making sense of missense in Lynch Syndrome: The clinical perspective. Cancer Prevention and Research, 3, 1371-1374. doi:10.1158/1940-6207.CAPR-10-0204



Joanne Ngeow and Charis Eng. Population-based universal screening for Lynch Syndrome: Ready, set… how? (2013). Journal of Clinical Oncology, 21, (20), 2527-2529. Retrieved from http://jco.ascopubs.org/content/31/20/2527.full

OSUhumangenetics. (November 7, 2012). Heather Hampel discusses Lynch Syndrome. http://www.youtube.com/watch?v=k_07Be7p2_8






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