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Abstracts Poster Abstracts - Dr Falk

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3. One pancreatic cancer case with at least 3 cases of breast cancer or onecase of ovarian cancer and one or more cases of breast cancer.4. Ashkenazi Jewish ancestry with one pancreatic cancer case and a singlecase (or more) of breast or ovarian cancer at any age in a first orsecond-degree relative (testing for the three Ashkenazi Jewish foundermutations may be sufficient).Peutz-Jeghers Syndrome (PJS) Definition:Individuals affected with Peutz-Jeghers syndrome have multiple gastrointestinalhamartomatous polyps and mucocutaneous pigmentation. Individuals with PJS are atincreased risk for developing pancreas, colon, breast, endometrial, ovarian, lung, ortestes.Relative Risk of Pancreatic Cancer:Up to 100 foldGene and Chromosomal Location:STK11/LKB1 on 19p13.3Individuals should be referred for genetic counseling to identify a potential geneticpredisposition to pancreatic cancer if:The individual can be clinically diagnosed with PJS based on the following:1. An individual has two or more histologically verified Peutz-Jeghers polyps,small bowel polyposis, and mucocutaneous hyperpigmentation, or2. An individual has small bowel polyposis, mucocutaneous hyperpigmentation,and a family history of PJS.Hereditary Non-Polyposis Colorectal Cancer Syndrome (HNPCC) Definition:HNPCC is a condition that is characterized by an increased risk of colon cancer andother cancers that include cancers of endometrium, ovary, stomach, small intestines,hepatobiliary tract, upper urinary tract, brain, and skin. Individuals diagnosed withHNPCC have an approximate 80% chance of developing colon cancer in theirlifetime. The average age of onset for colorectal cancer in these individuals is 44years. Women diagnosed with HNPCC have a 20-60% chance of developingendometrial cancer in their lifetime with an average of diagnosis of 46 years.Affected Gene: MLH1 and MSH2Relative Risk of Pancreatic Cancer: 1.5 foldIndividuals should be referred for genetic counseling to identify a potential geneticpredisposition to pancreatic cancer if:A clinical diagnosis of HNPCC can be based on one of the following 2 criteria.32

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