Bannayan-Riley-Ruvalcaba Syndrome
A Guide for patients and their families
Associated Cancer Risks
Sarah Burton, UI Health Care
Joy Larsen Haidle, MS, CGC, UI Health Care
Heather Hampel, MS, CGC, Ohio State University
Peer Review Status: Internally reviewed by the authors
First Published: 2002
Last Revised: 2002
Bannayan-Riley-Ruvalcaba Associated Cancer Risks
Individuals with Bannayan-Riley-Ruvalcaba or 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 BRR or Cowden syndrome, 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 BRR or Cowden syndrome is 38 years; however, cancer has been reported in patients ranging from14 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 BRR 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 BRR 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).
Cancers of the endometrium, kidney, skin, colon, small bowel, and ovary have been reported in individuals diagnosed with Bannayan-Riley-Ruvalcaba or Cowden's syndrome, but a true association between these syndromes 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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