Managing Patients with Lynch Syndrome or MYH-associated Polyposis (MAP)

Oncea diagnosis of Lynch syndrome is confirmed, the following medical management options may help reduce cancer risk or detect cancer at an earlier, more treatable stage or even prevent it.*

Lynch syndrome Management

Colon Cancer

Increased Surveillance for Colorectal Cancer

  • Colonoscopy ever 1-2 years beginning between age 20 and 25, OR 10 years before the earliest age of a patient’s family member diagnosed with colorectal cancer – whichever comes first50
  • Consider annual colonoscopy after age 4051,52,53
  • For MSH6 mutation carriers consider initiating colonscopy screending at age 30 – 35 or 10 years before the earliest age of a patient’s family member diagnosed with colorectal cancer.  This is due to the later average age of onset in MSH6 mutation carriers50

Surgical Management of Colorectal Cancer

  • If colon cancer is diagnosed (or more than one advanced adenoma is found) in a patient with Lynch syndrome, total colectomy with ileorectal anastomosis OR hemicolectomy is an option50,54
  • In patients unwilling or unable to undergo periodic colonscopy screening, prophylactic total colectomy with ileorectal anastomosis may be an option based on carrier status alone50,51,54

Endometrial and Ovarian Cancer

Surveillance for Endometrial and Ovarian Cancer

  • Consideration of referral to a gynecologic oncologist to discuss screening options with can include gynecologic exam, transvaginal ultrasound, endometrial aspiration and CA-125 every year, beginning between age 25 and 3550,51,52,55,56

Surgical Management of Endometrial and Ovarian Cancer

  •  Prophylactic total abdominal hysterectomy and bilateral salpingo-oophorectomy is a risk reducing option for women who have completed childbearing
  •  May also be considered at time of colon surgery if postmenopausal or childbearing is complete50

Surveillance for Other Lynch Syndrome-Related Cancers

  • For gastric and duodenal cancer: Consider upper GI endoscopy (wide side viewing scope) at age 25 – 30 years and repeat every 1 to 3 years depending on findings
  • For urothelial cancer: Consider urinalysis on an annual basis
  • For CNS cancer: Physical examination on an annual basis

MYH-Associated Polyposis (MAP):

  • For patients who have MAP, medical society management recommendations include colonoscopies every 1-2 years starting at age 25-30, upper endoscopies every 3-5 years starting at age 30-35, and surgical considerations. The most appropriate medical management will vary based upon your patient’s clinical presentation.282
  • For MYH mutation carriers (1 mutation), medical management should be determined by clinical findings and personal and family history of colorectal polyps and/or cancer. Current data are limited but suggest that any increase in risk, if present, is likely to be small.283

^ Lynch syndrome and MYH-associated polyposis (MAP) management is an evolving area. The most up to date management guidelines can be found at National Comprehensive Network. See the Colorectal Cancer Screening guidelines.. www.nccn.org

* Any discussion of medical management options is for general information purposes only and does not constitute a recommendation. While genetic testing and medical society guidelines provide important and useful information, medical management decisions should be made based on consultation between each patient and his or her healthcare provider.