Cowden Syndrome
A Guide for patients and their families
Cowden Syndrome Associated Cancer Risks
Sarah Burton, UI Health Care
Joy Larsen Haidle, MS, CGC, UI Health Care
Heather Hampel, MS, CGC, Ohio State University
Charis Eng, MD, PhD, Ohio State University
Peer Review Status: Internally reviewed by the
authors
First Published:
Last Revised: April 2002
Individuals with Cowden syndrome have an
increased risk for both benign and malignant tumor formation. The
breast and thyroid are the two most common sites for cancer
development. Of all the women diagnosed with CS, 30 to 50 percent of
patients will develop breast cancer and 50 to 70 percent of patients
will not develop breast cancer. The average age of breast cancer in
women with CS is 38 years; however, cancer has been reported in
patients ranging from 14 to 65 years of age. Women are also at
increased risk for other benign breast conditions, such as ductal
hyperplasia, intraductal papillomatosis, lobular hypertrophy,
fibrocystic breast disease, or fibroadenomas.
Although the risks are lower, men with CS also
have an increased risk for developing breast cancer. However, the
degree of risk for breast cancer in men is unknown. Thyroid adenomas,
multinodular goiter, and cancer are also seen at increased frequency
in individuals diagnosed with CS syndrome. Affected individuals have
a three to 10 percent lifetime risk for developing thyroid cancer
(and a 90 to 97 percent chance of not developing thyroid cancer).
Studies in the last few years have strongly suggested that
endometrial cancer is a true component of CS, although rigorous
studies with many patients have yet to be performed.
Cancers of the colon, skin, kidney, small
bowel, stomach, ovary, and lung have been reported in individuals
diagnosed with Cowden syndrome, but a true association between CS and
the risk for cancer has not been proven. Because these risks are not
clearly known, surveillance is an important part of your health care
program.
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