Paget's Disease of the Breast Health Article

Prevention

There are no specific factors that increase a person's risk of developing Paget's disease. Men who are at an increased risk of developing breast cancer include those who have had radiation exposure and those with Klinefelter's syndrome. Women's risk factors for breast cancer include:

• a personal history of breast cancer
• a family history of breast cancer
• alterations in certain genes (e.g. BRCA1 and BRCA2)
• changes in breast tissue (e.g. lobular carcinoma in situ or atypical hyperplasia)
• long-term exposure to estrogen (e.g. early age at first menstruation or late menopause), and possibly use of hormone replacement therapy
• exposure to diethylstilbestrol (DES) before birth
• first pregnancy after 30 years of age
• alcohol consumption

Regularly scheduled screening mammograms are recommended for all women over the age of 40 years. Those with a significant family history (one or more first-degree relatives who have been treated for breast cancer), should start annual mammograms 10 years younger than the youngest relative was when she was diagnosed, but not earlier than 35. Monthly breast self examinations and yearly clinical breast examinations are recommended for all women. Daily exercise, totalling two to four hours a week, decreases a woman's risk of breast cancer by 50% to 75%. Women with a high risk of breast cancer may take the drug tamoxifen, which has been shown to reduce the occurrence (or recurrence) of breast cancer. Women at a very high risk may choose to have a mastectomy to prevent breast cancer (prophylactic mastectomy).

Special concerns

Of special concern to the young woman with breast cancer is the impact that treatment will have on her fertility and body image. Depression is common. There is ongoing research investigating whether timing breast cancer surgery to coincide with the luteal phase (after ovulation) of the menstrual cycle leads to an increased survival rate.

Resources

BOOKS

Bruss, Katherine, Christina Salter, and Esmeralda Galan, eds. American Cancer Society's Guide to Complementary and Alternative Cancer Methods. Atlanta: American Cancer Society, 2000.

Kronenberg, Fredi, Patricia Murphy, and Christine Wade. "Complementary/Alternative Therapies in Select Populations: Women." In Complementary/Alternative Medicine: An Evidence-Based Approach, ed. John Spencer and

Joseph Jacobs. St. Louis: Mosby, 1999, pp.340-62. Jatoi, Ismail, ed. The Surgical Clinics of North America: Breast Cancer Management, ed. Philadelphia: W. B. Saunders Company, 1999.

Lemon, Henry. "Cancer of the Female Breast." In Current Therapy in Cancer, ed. Foley, John, Julie Vose, and James Armitage. Philadelphia: W. B. Saunders Company, 1999, pp.109-15.

PERIODICALS

Lloyd, J. and A. M. Flanagan. "Mammary and Extramammary Paget's Disease." Journal of Clinical Pathology 53 (Octo ber 2000): 742-49.

ORGANIZATIONS

American Cancer Society. 1599 Clifton Road NE, Atlanta, GA 30329. (800) ACS-2345. <http://www.cancer.org>.

Cancer Research Institute, National Headquarters. 681 Fifth Ave., New York, NY 10022. (800) 992-2623. <http://www.cancerresearch.org>.

National Alliance of Breast Cancer Organizations. 9 East 37th St., 10th Floor, New York, NY 10016. (888) 806-2226. <http://www.nabco.org>.

National Institutes of Health. National Cancer Institute. 9000 Rockville Pike, Bethesda, MD 20982. Cancer Information Service: (800) 4-CANCER. <http://cancernet.nci.nih.gov>.

Y-Me Advocacy Program. 212 West Van Buren St., 5th Floor, Chicago, IL 60607. (312) 986-8338. <http://www.y-me.org>.

Belinda Rowland, Ph.D.

Areola

—The darkened area that surrounds the nipple.

Diethylstilbestrol (DES)

—A medication used between 1945 and 1970 to prevent miscarriage.

Extramammary Paget's disease

—Paget's disease that is located anywhere on the body, excluding the breasts.

Luteal phase

—That part of the menstrual cycle that begins after ovulation and ends at menstruation.

Mastectomy

—Surgical removal of breast tissue. Mastectomy may be partial, when only some tissue is removed, or radical, when all breast tissue and adjacent tissues are removed.

QUESTIONS TO ASK THE DOCTOR

• What type of cancer do I have?
• What stage of cancer do I have?
• What is the five-year survival rate for women with this type and stage of cancer?
• Has the cancer spread?
• What are my treatment options?
• How much breast tissue will you be removing?
• Where will the scars be?
• What will my breast look like after surgery?
• When can I have breast reconstruction?
• What are the risks and side effects of these treatments?
• What medications can I take to relieve treatment side effects?
• Are there any clinical studies underway that would be appropriate for me?
• What effective alternative or complementary treatments are available for this type of cancer?
• How debilitating is the treatment? Will I be able to continue working?
• Are there any local support groups for breast cancer patients?
• What is the chance that the cancer will recur?
• Is there anything I can do to prevent recurrence?
• How often will I have follow-up examinations?