$Unique_ID{BRK03553} $Pretitle{} $Title{Cancer, Breast} $Subject{Cancer Breast Carcinoma of the Breast Cancer of the Breast Female Breast Cancer Male Breast Cancer Inflammatory Breast Cancer Benign Tumors of the Breast Benign Breast Disease Mastitis Paget's Disease of the Breast} $Volume{} $Log{} Copyright (C) 1990, 1991 National Organization for Rare Disorders, Inc. 807: Cancer, Breast ** IMPORTANT ** It is possible that the main title of the article (Breast Cancer) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Carcinoma of the Breast Cancer of the Breast Disorder Subdivisions: Female Breast Cancer Male Breast Cancer Inflammatory Breast Cancer Information on the following disorders can be found in the Related Disorders section of this report: Benign Tumors of the Breast Benign Breast Disease Mastitis Paget's Disease of the Breast General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Breast Cancer is one of approximately 200 different types of cancer. Cancer is a disease in which abnormal cell development occurs, causing destruction of healthy cells. Breast Cancer is one of the most common forms of cancer. Generally, breast cancer is discovered by the patient or physician as a hard, irregularly-shaped mass within the breast. It can also be detected by mammogram (breast x-ray). When detected early, breast cancer is treatable. People who have first degree relatives with breast cancer are at increased risk and should enter a breast cancer screening program on a continuous basis. Symptoms Breast Cancer is usually discovered by the patient or physician as a painless, usually hard lump in the breast. In rare cases, pain and tenderness may also occur. Other symptoms may include changes of the breast such as the shape, contour, swelling, thickening, skin dimpling, or pore enlargement. Retraction or scaliness of the nipple may also occur. Bloody discharges occur in about 33% of patients with breast cancer and in about 30% of patients with a benign (noncancerous) condition; discharges from the nipple that are not bloody are usually not related to cancer. In inflammatory breast cancer, the breast is inflamed and swollen, and its surface dimples like the surface of an orange peel (peau d'orange). There is a rare form of male breast cancer. The symptoms are generally the same as those found in female breast cancer. Male breast cancer accounts for 1% of all malignant breast carcinomas. Causes Like most forms of cancer, the exact cause of breast cancer is not known. Some risk factors that are statistically linked with breast cancer are hormonal effects such as early menstruation, late menopause, first pregnancy after the age of 40, or a family history of the disease (especially in first- degree relatives such as mothers and sisters). Other risk factors are age (two-thirds of breast cancer patients are post-menopausal women), living in a Western society (such as the United States, possibly because of diet), and possibly a diet high in fat and dairy products or high consumption of alcohol. In men who have Klinefelter's Syndrome (or other metabolic changes of estrogen levels in males), or idiopathic gynecomastia (excessive development of the male breast), there is an increased risk of developing male breast cancer. (For more information choose "Klinefelter" as your search term in the Rare Disease Database). Researchers have developed a mouse with the genetic form of breast cancer. Breeding these mice may lead to enhanced understanding and treatment of breast cancer. Scientists are using genetic mapping in hopes of finding the way that inherited forms of breast cancer may develop. A gene located on the long arm (q) arm of chromosome 17, BRCA1, is suspected of being responsible for inherited forms of breast cancer. Some researchers at Utah Medical Center are suggesting Proliferative Breast Disease (PBD), a benign multiplication breast tissue which is an inherited condition, is often present in women who later develop breast cancer Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother. Breast cancer can be inherited as an autosomal dominant trait, and there may be a recessive form of breast cancer as well. In dominant disorders a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is fifty percent for each pregnancy regardless of the sex of the resulting child. In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If one receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will show no symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is twenty-five percent. Fifty percent of their children will be carriers, but healthy as described above. Twenty-five percent of their children will receive both normal genes, one from each parent, and will be genetically normal. Affected Population Breast Cancer affects about 130,000 women in the United States annually, and usually occurs between the ages of 35 and 65. Although very rare, breast cancer occurs in males at 1% of the rate found in females. Related Disorders Often, other types of tumors can be similar to the malignant tumors of breast cancer. Most lumps found in the breast are benign tumors or cysts (fibroadenomas, papillomas). Benign (noncancerous) breast disease (mammary dysplasia) such as cystic disease (lumpy breasts) or inflammatory diseases such as mastitis (inflammation of the breast) or fat necrosis may mimic symptoms of breast cancer. In Paget's Disease of the Breast there are changes in the nipple such as scaliness, oozing or crusting, and patches on the nipple and/or areola. This is caused by an underlying cancer (usually an adrenocarcinoma). Diagnosis is made by skin biopsy. (For more information on this disorder, choose "Paget's Disease of the Breast" as your search term in the Rare Disease Database). Therapies: Standard Generally, in diagnosing breast cancer, a mammogram (breast x-ray) is performed first. Thermography, another breast imaging technique, may be used in some cases but it is not as sensitive as mammography. Biopsy is made to confirm malignancy. If necessary, other tests may include a complete blood count, chest x-ray, and tests or scans for involvement of liver or bone. It is important to determine how far the disease has spread since this determines the appropriate treatment program. First, physicians determine what "stage" the disease is in. The stage depends on the number and location of malignant sites, and if the cancer has traveled. Treatment is based upon the individual; it varies from patient to patient. In general, the earlier breast cancer is diagnosed, the better the prognosis is. A mastectomy is the standard treatment for people with breast cancer. A segmental mastectomy (or lumpectomy) for removal of the lump and surrounding tissue may be all that is necessary, or removal of the entire breast and surrounding lymph nodes (found in the armpit) be required. Radical mastectomy is removal of the entire breast, lymph nodes and underlying chest muscle, and is rarely performed in recent years. For most patients, breast reconstruction or plastic surgery can correct any disfigurements left by the surgery. The decision on the type of treatment is left to the patient and the surgeon. Radiation therapy (radiotherapy) and/or chemotherapy are treatment options for breast cancer. Radiation may be used to destroy cancer cells. Chemotherapy involves the use of "anticancer" drugs. There are several drug combinations now in use such as CMF (cyclophosphamide, methotrexate, and 5- fluorouracil), or CMFVP (CMF plus vincristine and prednisone). All these drugs may produce adverse side effects and must be carefully monitored by a doctor. Endocrine therapy, also called hormone manipulation, benefits about 30% of breast cancer patients. Other treatment is symptomatic and supportive. Therapies: Investigational Another breast imaging technique used to diagnose breast cancer is sonography, or "ultrasound," (the use of high-frequency sound waves to form special pictures) which is still in experimental stages. Two hormonal threrapies are being tested by the FDA for treatment of metastatic breast cancer. They are sponsored by Adria Laboratories, Inc., P.O. Box 16529, Columbus, OH 43216-6529. They are titled 6- methylenandrosta-1, 4-Diene 3, 17-Dione and Toremifene. The National Cancer Institute conducts clinical trials on new drugs being tested for breast cancer and other forms of cancer. To learn about locations of these investigations, contact the Cancer Information Service listed in the Resources section of this report. Autologous Bone Marrow transplants (ABMT) are being studied as a treatment for Metastic Breast Cancer. A study of ABMT, printed in the April 15, 1992 issue of the medical journal JAMA, revealed ABMT increased life expectancy by an average of six months in these patients at a cost of $115,800 per year of life gained. Twenty percent of women who underwent ABMT lived five years after the procedure and were still in remission. Three percent of women died as a direct result of the treatment. This disease entry is based upon medical information available through April 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Breast Cancer, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Y-ME 18220 Harwood Ave. (800) 221-2141 (708) 799-8228 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) References CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 1452-1458. INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1112-1120. MENDELIAN INHERITANCE IN MAN, 8th Ed.: Victor A. McKusick; Johns Hopkins University Press, 1986. Pp. 114, 843. WORLD BOOK MEDICAL ENCYCLOPEDIA: Erich E. Brueschke, M.D., et al, eds; World Book, Inc., 1988. Pp. 147. Research Resources Reporter, National Institutes of Health, June, 1991. P. 5. EFFICACY AND COST EFFECTIVENESS OF AUTOLOGOUS BONE MARROW TRANSPLANTATION IN METASTIC BONE CANCER., Hillner, et al., JAMA, April 15, 1992, (vol 267, No. 15)). Pp. 2055-2061.