Preventive Surgery

Colon

Recommendations from the National Comprehensive Cancer Network, (NCCN),  a NIH research consortium considers subtotal colectomy as an option for persons with Lynch Syndrome who have adenomatous polyps and those who are not willing or unable to undergo periodic colonic surveillance. The thought process is by removing 30% of the colon the overall risk is decreased 30%. Likewise if 2/3 of the colon is removed the risk is reduced by 2/3. So a subtotal colectomy, which removes about 90% of the colon , should reduce the risk to just 10% of baseline. Also, this allows a screening flexible sigmoidoscopy to be utilized instead of a full colonoscopy. This should increase screening follow-up rates and decrease costs. Since a sigmoidoscopy does not always require anesthesia, besides overall cost, recovery time and surgical are also reduced.  A recent article, "Deciding the operation type according to mismatch repair status among hereditary nonpolyposis colorectal cancer patients: should a tailored approach be applied, or does one size fit all?", published in Hereditary Cancer in Clinical Practice backed up this hypothesis showed an improved disease free survival for lynch patients comparing extended colectomy vs a segmental colectomy.

Uterine

In 2006 an excellent article,"Prophylactic surgery to reduce the risk of gynecologic cancers in the Lynch syndrome," was published in the New England Journal of Medicine. This study showed a significant risk reduction by utilizing  a preventative hysterectomy with bilateral salpingo-oophorectomy.  The control group had a 33% incidence of endometrial cancer and 5% incidence of ovarian cancer, compared to no endometrial or ovarian cancer in the TAH BSO group.  Based on this study and several others, the National Comprehensive Cancer Network, NCCN, recommends a hysterectomy and bilateral salpingo-oophorectomy be presented as an option to female patients, with a discussion of the risks and benefits. It is advised to have surgery after childbearing is completed or after the age of 40. However, if the family history is significant at an earlier age or if testing requires surgery prior to the completion of childbearing, it is important to consider egg cryopreservation.

Fertility

One of the biggest concerns people have is passing a hereditary disease, like Lynch syndrome to their children. Because Lynch syndrome is autosomal dominant, there is a 50% chance of passing this on to offspring. To avoid this risk, Preimplantation Genetic Testing for Monogenic Diseases (PGT-M) can be utilized. Once a polymorphism or Lynch causing gene is identified, reproductive specialists can identify affected genes in the embryos created during an In-vitro fertilization cycle. By having this information, embryos not affected by the condition can be selected and be transferred to patients yielding a normal pregnancy and an unaffected baby, therefore cutting the Lynch hereditary transmission. Albeit some fertility treatments like this can sometimes be expensive or present limitations in access to care, PGT-M it is one of the best options to date in order to prevent the transmission of Lynch syndrome. These technologies shed some light and promise for the future of reproductive choices in patients with Lynch and should be broadly discussed at the onset of a diagnosis for Lynch syndrome.

In Vitro Fertilization