Extracolonic Manifestations of Hereditary Nonpolyposis Colorectal Cancer (HNPCC)

The uterus is the second most common site of cancer in women with HNPCC: More than 40 percent will develop it. In some families, it is more common than colon cancer.

  • Recommendation: Women need a Pap test and pelvic ultrasound every one to two years beginning at age 25. If uterine cancer runs in the family, a hysterectomy should be considered. If there is surgery for a colon cancer, the uterus and ovaries should probably be removed at the same time

Ovarian cancer is more common in women with HNPCC than in the general public, although it tends to be less common than uterine cancer and less likely to run in families.

  • Recommendation: Have a pelvic ultrasound every one to two years, beginning at age 25.

Urinary System
HNPCC patients are at increased risk for cancer of the parts of the urinary system that hold urine, e.g., the kidneys collecting systems, ureters and lining of the bladder. Even though the risk is more-than-average, urinary system cancers are rare.

  • Recommendation: If urinary system cancers run in the family, have urine checked once a year for malignant cells.

Stomach cancer is associated with HNPCC, but the risk is not very high. If stomach cancer runs in the family, patients should be checked.

  • Recommendation: Undergo gastroscopy every two to three years if gastric or small bowel cancer runs in the family.

Small Intestine
As with stomach cancer, there is a small increased risk of small bowel cancer.

  • Recommendation: If small bowel or gastric cancer is in the family, check every two to three years with regular endoscopy or capsule endoscopy.

Other Organs
A number of other organs including gallbladder, liver, pancreas and brain may develop cancer in HNPCC families. However, these types of cancer don't occur often enough to warrant routine checks.

Reviewed by a Cleveland Clinic medical professional.

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