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EPCAM: Risk Management
Read about different genes that are linked to hereditary cancer, their associated risks and guidelines for screening, preventing and treating cancers in people with inherited mutations in these genes.

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Risk Management for People with an Inherited Mutation

People with mutations have options for managing their increased cancer risk. Experts at the National Comprehensive Cancer Network (NCCN) created guidelines for people with an mutation to manage their cancer risk.

We recommend that you speak with a genetics expert who can look at your personal and family history of cancer and can help you decide on a plan for managing your risk. 

NCCN recommends that all people with an inherited mutation receive education on the signs and symptoms of Lynch syndrome-related cancers. NCCN guidelines for specific cancers are listed below. You can learn more about risk management options in our section on Screening and Risk Reduction by Cancer Type

NCCN risk management guidelines for people with mutations include information on the following cancers:

 

People with an  mutation may also qualify for clinical trials looking for more effective screening or prevention for cancer.


Colorectal cancer

Beginning Age

Recommendation

20-25 (or 2-5 years before the earliest age of colon cancer in the family, if diagnosed before age 25)

High quality colonoscopy every 1-2 years. Speak with your doctor about whether your screenings should be yearly or every two years. Men, people over age 40, and people with a personal history of colon cancer or colon polyps may benefit most from yearly screenings. 

No set age

Daily aspirin can decrease the risk for colorectal cancer. The best dose and timing for aspirin is not known. Speak with your doctor about the benefits and risks, best timing and dose.

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Endometrial cancer

Beginning Age

Recommendation

Additional Information

No set age

Endometrial cancer can often be caught early based on symptom awareness followed by a biopsy. Learn to be aware of the symptoms of endometrial cancer and immediately report any symptoms to your doctor. Keep a diary of menstrual cycles in order to detect any changes in bleeding.

Symptoms of endometrial cancer include:

  • unusual vaginal bleeding
  • pelvic or abdominal pain
  • bloating or distended belly
  • difficulty eating
  • increased urination or pressure to urinate

30-35

Discuss the benefits, risks and costs of an endometrial biospsy every 1-2 years with your doctor. 

Endometrial cancer screening does not have proven benefit in individuals with Lynch syndrome. However, endometrial biopsy is an accurate test for finding cancer.

After menopause

Discuss the benefits, risks and costs of regular endometrial cancer screening through transvaginal ultrasound.

Endometrial cancer screening does not have proven benefit in individuals with Lynch syndrome. Despite this, some people prefer to undergo regular screening through transvaginal ultrasound.

After completion of child-bearing

Discuss the benefits, risks and costs of a risk-reducing hysterectomy (surgical removal of the uterus). 

Total hysterectomy lowers the risk, but it has not been shown to reduce death from endometrial cancer. 

No specified age

Speak with your doctor about the benefits, risks and timing of oral contraceptives. 

Speak with your doctor about the benefits, risks and timing of oral contraceptives. 

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Ovarian and  cancer

Beginning Age

Recommendation

Additional Information

No set age

Be aware of ovarian cancer symptoms. Report any of the following symptoms that persist for several weeks and are a change from normal to your doctor.

Routine ovarian cancer screening using transvaginal ultrasound and a CA-125 blood test has not shown benefit and is not recommended.

Symptoms of ovarian cancer include:

  • pelvic or abdominal pain
  • bloating or distended belly
  • difficulty eating
  • feeling full sooner than normal
  • increased urination or pressure to urinate 

After completion of child-bearing

Speak with your doctor about the benefits, risks and timing of risk-reducing removal of ovaries and fallopian tubes (RRSO). 

No set age

Speak with your doctor about the benefits and risks of oral contraceptives for lowering ovarian cancer risk. 

Oral contraceptives (birth control pills) may lower the risk for ovarian cancer in people with Lynch syndrome.

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Pancreatic cancer

There are two tests that are used to look for pancreatic cancer in high-risk people. 

  • MRCP (Contrast-enhanced magnetic resonance cholangiopancreatography) is a special type of  imaging that looks closely at the pancreas, liver, gallbladder, bile duct and pancreatic duct to find abnormalities such as cancer.
  • EUS (Endoscopic ) involves passing a tiny scope with an attached  probe down the esophagus to the stomach. This allows doctors to look closely at the pancreas.  

Beginning Age

Recommendation

50 (or younger based on family history) 

For EPCAM mutation carriers with a first- or second- degree relative with pancreatic cancer

  • Discuss the benefits, risks, costs and limitations of screening with your doctor.
  • Screening should include annual MRCP or EUS or both. 
  • Screening should be performed in a facility with experience in screening high-risk patients for pancreatic cancer. 

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Bladder, kidney and ureteral cancer

Beginning Age

Recommendation

30-35

For EPCAM mutation carriers with a relative with bladder, kidney or ureteral cancer

  • Discuss the benefits, risks, costs and limitations of screening with your doctor.

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


 cancer

Beginning Age

Recommendation

40

Have a conversation with your doctor about the potential benefits, risks, costs and limitations of screening for prostate cancer. 

  • If you choose to have screening, the guidelines recommend an annual digital rectal exam and Prostate Specific Antigen (PSA) test. 

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Stomach cancer

Beginning Age

Recommendation

30-40 (or younger based on family history)

Begin stomach cancer screening using a procedure known as EGD  (esophagogastroduodenocopy) and repeat every 2-4 years. 

  • More frequent screening may be recommended based on prior screening findings. 
  • If gastric screening is performed, considered random biopsy to test for H. pylori. If gastric biopsy is not performed, consider non-invasive testing for H. pylori.
    • People who test positive should receive treatment for H. pylori. 

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Brain cancer

Beginning Age

Recommendation

No set age

Learn the signs of brain cancer and report any symptoms to your doctor.

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Skin cancer

Beginning Age

Recommendation

No set age

Talk with your doctor about the benefits of having a skin exam every 1-2 years with an expert trained in finding skin changes related to Lynch syndrome

Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Colorectal, vs. 2 2023


Other cancers

The guidelines do not address screening or prevention of other cancers for people with  mutations. However, clinical trials may be available.


Last updated June 22, 2024