Lynch Syndrome dramatically increases the risk of colon cancer. The lifetime risk estimates are 54-74% for men and 30-52% for women for MLH1 and MLH2 gene mutation carriers and 44-69% for men and 20-30% for women for MSH6 carriers.
Lynch Syndrome also increases the risk of other cancers. The most common is endometrial with a 42% to 71% lifetime risk. This includes endometrial adenocarcinoma, clear cell carcinoma, uterine papillary serous carcinoma and malignant mixed Mullerian tumors. Lynch Syndrome is also associated with cancers of the stomach (6-9%), small intestine (3-4%), hepatobiliary (1%), brain (2-3%), ovary (6-12%), ureter and renal pelvis (3-8%). Can also add pancreas (1-4%) and sebaceous gland neoplasms (1-9%).
Risk of Cancers by Subtype
Each of the subtypes, MLH1, MSH2, PMSH6, and PMS2 has a different lifetime risk of cancers. This also varies by sex. To help determine the specific risk for an individual a large database of Lynch syndrome patients has been created. This is known as the Prospective Lynch Syndrome Database (PLSD). Fortunately, this information is available free of charge at www.plsd.edu
The PLSD website shows the cumulative risk for each specific subtype (MLH1, MSH2, PMSH6, and PMS2). It also shows specific cancer risks by organ (colon, endometrium, ovaries, prostate, urinary bladder, kidney/ureter, stomach/intestine/ pancreas, breast, and brain) and subtype. The data is further broken down if there was or was not a previous cancer diagnosed in the individual. The PLSD website is very easy to use and provides good data that is being updated on a regular basis.