Skip Navigation LinksManagement Recommendations

The following are current gene-specific medical management recommendations for individuals Lynch syndrome. Of note, other than the recommendations for colon and endometrial cancer, these recommendations are based on expert opinion rather than evidence-based.

MLH1MSH2, EPCAM

Surveillance

  • Colonoscopy with polypectomy beginning at age 20-25
    • If a relative was diagnosed with colon cancer prior to age 25, begin colonoscopy 2-5 years prior to that relative's diagnosis.
    • Repeat every 1-2 years
  • Annual physical examination including neurologic examination beginning at age 25-30
  • Consider EGD with extended duodenoscopy beginning at age 40 years
    • Repeat every 3-5 years
  • Consider annual dermatological exams
  • Consider annual pancreas cancer imaging (alternating between EUS and MRI) if family history includes close relative(s) with pancreas cancer
    • Begin at age 50 or 10 years earlier than the youngest diagnosis of pancreatic cancer in the family, whichever is earliest.
  • Consider yearly urinalysis beginning at age 30-35
  • Consider yearly prostate cancer screening begining at age 40 with PSA blood test

Risk Reduction
  • Women: Consider prophylactic hysterectomy and bilateral salpingo-oophorectomy (removal of the uterus, ovaries, and fallopian tubes) when childbearing is complete.
    • If not pursuing these risk-reducing surgeries:
      • Be aware of and report dysfunctional uterine bleeding, abdominal or pelvic pain, bloating, difficulty eating, and/or increased urinary frequency or urgency if symptoms persist for several weeks
      • Consider yearly endometrial sampling beginning at age 30-35, every 1-2 years
      • Consider use of hormonal birth control for risk reduction
  • Consider taking daily aspirin for colon cancer risk reduction
  • Consider H.pylori testing and treating if detected

MSH6

Surveillance
  • Colonoscopy with polypectomy beginning at age 30-35
    • If a relative was diagnosed with colon cancer prior to age 25, begin colonoscopy 2-5 years prior to that relative's diagnosis.
    • Repeat every 1-2 years
  • Annual physical examination including neurologic examination beginning at age 25-30
  • Consider EGD with extended duodenoscopy beginning at age 40 years
    • Repeat every 3-5 years
  • Consider annual dermatological exams
  • Consider annual pancreas cancer imaging (alternating between EUS and MRI) if family history includes close relative(s) with pancreas cancer
    • Begin at age 50 or 10 years earlier than the youngest diagnosis of pancreatic cancer in the family, whichever is earliest.
  • Consider yearly urinalysis beginning at age 30-35
  • Consider yearly prostate cancer screening begining at age 40 with PSA blood test

Risk Reduction
  • Women: Consider prophylactic hysterectomy and bilateral salpingo-oophorectomy (removal of the uterus, ovaries, and fallopian tubes) when childbearing is complete.
    • If not pursuing these risk-reducing surgeries:
      • Be aware of and report dysfunctional uterine bleeding, abdominal or pelvic pain, bloating, difficulty eating, and/or increased urinary frequency or urgency if symptoms persist for several weeks
      • Consider yearly endometrial sampling beginning at age 30-35, every 1-2 years
      • Consider use of hormonal birth control for risk reduction
  • Consider taking daily aspirin for colon cancer risk reduction
  • Consider H.pylori testing and treating if detected
PMS2

Surveillance

  • Colonoscopy with polypectomy beginning at age 30-35
    • If a relative was diagnosed with colon cancer prior to age 25, begin colonoscopy 2-5 years prior to that relative's diagnosis.
    • Repeat every 1-2 years
  • Annual physical examination including neurologic examination beginning at age 25-30
  • Consider EGD with extended duodenoscopy beginning at age 40 years
    • Repeat every 3-5 years
  • Consider annual dermatological exams
  • Consider yearly urinalysis beginning at age 30-35
  • Consider yearly prostate cancer screening begining at age 40 with PSA blood test

Risk Reduction
  • Women: Consider prophylactic hysterectomy and bilateral salpingo-oophorectomy (BSO: removal of the uterus, ovaries, and fallopian tubes) when childbearing is complete. Notably, there is insufficient evidence to support the utility of BSO.
    • If not pursuing these risk-reducing surgeries:
      • Be aware of and report dysfunctional uterine bleeding, abdominal or pelvic pain, bloating, difficulty eating, and/or increased urinary frequency or urgency if symptoms persist for several weeks
      • Consider yearly endometrial sampling beginning at age 30-35, every 1-2 years
      • Consider use of hormonal birth control for risk reduction
  • Consider taking daily aspirin for colon cancer risk reduction
  • Consider H.pylori testing and treating if detected

Resources

Patient factsheet about Lynch Syndrome.

2020 Provider Education Presentations

Presenter:
 Priyanka Kanth, MD, MSCI, FACG 
Talk: Surveillance Recommendations

 


Presenter: Joanne Jeter, MD
Talk: Chemoprevention in Lynch Syndrome
 


Presenter: Jessica Cohan, MD, MAS
Talk: Gastroinestinal Surgerical Considerations with Lynch Syndrome

 


Presenter: Rob Dood, MD, MSCE
Talk: Gynecological Risk Reduction in Lynch Syndrome

 



Pancreatic Cancer Surveillance (2021 Updated Presentations)

Presenter: Joanne Jeter, MD, MS
Talk: High Risk Pancreatic Cancer Surveillance


 

Presenter: Kathyrn R. Byrne, MD
Talk: Use of Endoscopic Ultrasound to diagnose PDAC in high risk patients

 


Presenter: Rebecca Y. Kim, MD, MPH, FACS
TalkHereditary Pancreatic Cancer: A Surgeon's Perspective