Cowden Syndrome
A Guide for patients and their families
What Should I Be Doing About This Condition?
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
There are many screening recommendations that
people diagnosed with Cowden syndrome should follow. In many cases,
these proactive steps can help manage benign growths and detect any
cancers at an early stage when they are best treated. The recommended
screenings for CS patients are summarized in a table on the next page.
Because of the increased risk for breast
cancer, women should have increased breast cancer surveillance. This
includes performing monthly breast self-examination, getting a
clinical breast examination every 6 to 12 months beginning at 21
years of age, in addition to annual mammograms beginning at 30 to 35
years of age. Some young women have dense breast tissue that can
limit the detection of early breast cancer associated with CS.
Prophylactic mastectomy is an option available to women for whom
screening is not effective or who have had multiple breast biopsies.
Men also have an increased risk for breast cancer and because of this
should perform a monthly breast self-exam beginning at age 35 to 45
years. Women should also receive endometrial cancer screening
beginning at age 30 to 35 years. Premenopausal women should have
blind-repel biopsies annually. Post-menopausal women should have an
annual trans-vaginal ultrasound.
Both men and women should receive annual
thyroid cancer surveillance that includes a baseline ultrasound of
the thyroid during adolescence. After the baseline evaluation is
complete, annual thyroid palpation to look for any changes is
recommended. Because thyroid nodules can be a common feature,
experienced endocrinologists need to evaluate any thyroid nodules to
determine if they require additional follow-up, such as a
biopsy.
Skin and colon cancer screenings should also be
done annually. Yearly visits to a dermatologist can help manage the
CS skin findings and screen for skin cancers. There is no clearly
increased risk for colorectal cancer for patients with CS, but a
baseline colonoscopy should be done at age 50 years. If this screen
shows only hamartomas (a common feature), then annual fecal occult
blood screenings and sigmoidoscopies every three to five years or
colonoscopies every ten years are needed. The screening guidelines
are similar to the American Cancer Society guidelines for colon
cancer screening in the general population.
Because some experts who study CS believe kidney cancer can be part of CS, without much data, annual urinalysis should be considered to look for hidden blood in the urine.
Title Page
|
|
|
