$Unique_ID{BRK04077} $Pretitle{} $Title{Paget's Disease of the Breast} $Subject{Paget's Disease of the Breast Nipple Cancer Adenocarcinoma of the Nipple Paget's Disease of the Nipple Mammary Extramammary Paget's Disease Extramammary Paget's Disease Bowen's Disease Malignant Melanoma Psoriasis Mycosis Fungoides Eczema} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 787: Paget's Disease of the Breast ** IMPORTANT ** It is possible that the main title of the article (Paget's Disease of the Breast) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Nipple Cancer Adenocarcinoma of the Nipple Paget's Disease of the Nipple Mammary or Extramammary Paget's Disease Extramammary Paget's Disease Information on the following diseases can be found in the Related Disorders section of this report: Bowen's Disease Malignant Melanoma Psoriasis Mycosis Fungoides Eczema 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. Paget's Disease of the Breast is a very rare form of cancer. It is characterized by skin changes resembling eczema around the nipple and breast. It may also occur in the skin areas of the genitals and rectum. When it spreads to these areas of the body Paget's Disease of the Breast is called Extramammary Paget's Disease. Paget's Disease of the Breast often signals the existence of other internal cancer. It can occur in men as well as women. Paget's Disease of the Breast is not Paget's Disease of the bones. For more information about the type of Paget's Disease that effects the bones, choose "Paget's" as your search term in the Rare Disease Database. Symptoms Paget's Disease of the Breast is characterized by scaling, oozing and sharply defined inflamed patches on the skin of the nipple and areola of the breast. This disorder occurs in both men and women. It is nearly always associated with glandular (adenocarcinoma) cancer of the breast. It may or may not spread to the genital and rectal areas. If it does spread, Paget's Disease of the Breast is called Extramammary Paget's disease. When spreading occurs the Extramammary Paget's Disease is characterized by itchy, patchy, crusty areas with well defined borders that may include the lower abdominal wall, naval area, vagina, and rectum in women. In men the rectum, prostate, urethra and other parts of the genital and urinary tracts may be involved. When detected early and involving only the nipple area of the breast, the cure rate for Paget's Disease of the Breast is very high. However, when extramammary tissue is involved the treatment is more difficult. Causes The exact cause of Paget's Disease of the Breast and Extramammary Paget's Disease is not known. Since the disorder arises from skin (epidermotropic) cancer cells in the connective tissue layer (parenchyma) of the breast, some scientists believe that this type of cancer may be an early warning sign of more common breast cancers or other internal forms of cancer. Affected Population Paget's Disease of the Breast is a very rare from of cancer. It affects both males and females. However, the ratio is much higher in women than in men, and the age of onset is usually after the age of fifty. Related Disorders Symptoms of the following disorders can be similar to those of Paget's Disease Comparisons may be useful for a differential diagnosis: Bowen's Disease is a precancerous, slow growing skin malignancy. The major symptom is a red-brown, scaly or crusted patch on the skin which resembles psoriasis or dermatitis. It may occur on any part of the skin or in the mucous membranes. The patch is usually irregular in shape and causes a raised area which shows an oozing red surface when the crust is scratched or removed. Like many forms of skin cancer, chronic sun exposure may cause it to develop. (For more information on this disorder, choose "Bowen" as your search term in the Rare Disease Database). Malignant Melanoma is a common form of skin cancer. It is marked by tumors of the pigmentation cells (melanocyte) of the skin. These tumors may appear in different sizes, shapes, and shades of color, and may spread to adjacent parts of the body. Tumors may also spread through the blood and lymph circulation to other organs. Early treatment of malignant Melanoma is essential in order to cure the disease. (For more information on this disorder, choose "Malignant Melanoma" as your search term in the Rare Disease Database). Psoriasis is a common chronic and recurrent skin disorder characterized by dry, well-circumscribed silvery gray scaling spots or plaques which usually appear on the scalp, elbows, or knees. Symptoms may begin gradually. Psoriasis characteristically involves the scalp, extremities, the back and buttocks with sharply outlined lesions consisting of red spots or plaques covered with scales that usually do not itch. These lesions may heal without scarring and hair growth near the plaques is not affected. In severe cases, Psoriasis lesions may appear in pustular form. General health usually is not affected, unless severe arthritis (psoriatic arthritis) or untreatable scaling develops. However, stress may be caused by the stigma of an unsightly skin disorder. (For more information on these disorders, choose "Psoriasis" or "Psoriatic Arthritis" as your search terms in the Rare Disease Database). Mycosis Fungoides is a chronic progressive lymphocyte disorder arising in the skin. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells may occur. The disorder may spread to other parts of the body including the gastrointestinal system, liver, spleen, or brain. Itching, pain, skin plaques, fever, weight loss and anemia may occur. The cause is unknown. It is thought to be a malignant growth of lymph tissue originating in the skin. (For more information on this disorder, choose "Mycosis Fungoides" as your search term in the Rare Disease Database). Eczema is a very prevalent skin disorder characterized by red, itchy, non-contagious inflammation of the skin. It may be acute or chronic with red skin patches, pimples, crusts, or scabs occurring either alone or together. The skin may be dry or it may discharge a watery fluid, resulting in an itching or burning sensation. The affected skin may become infected. Eczema tends to occur in persons who have allergies. Therapies: Standard Treatment of Paget's Disease of the Breast involves determining exactly which type of Paget's is present. When there is disease of the nipple of the breast with no lymph nodes affected, surgery is usually performed to remove the affected area. Radiation and/or chemotherapy with or without surgery may also be necessary. If there is lymph node involvement as well as disease of the nipple, surgery may involve a simple or radical mastectomy and radiation treatment. If the Paget's is present because of other underlying cancer of the breast, then that cancer is treated first and the Paget' treatment is determined by the type of other cancer found. When the patient has Extramammary Paget's the treatment is determined by the organs affected. One of the most effective tools in diagnosing breast cancers is mammography. However, in many cases of Paget's Disease of the Breast no mass is present and a mammogram may not indicate presence of a malignancy. In this situation the nipple wedge biopsy may show Paget's Disease of the Nipple as well as intraduct carcinoma of the breast. Anti-Keratin antibody staining is also a very helpful technique used by physicians to determine the exact type of Paget's Disease of the Breast the patient has. Other treatment is symptomatic and supportive. Therapies: Investigational Research on all types of breast cancer is being pursued at the National Cancer Institute. For information about investigational therapies and clinical trials contact the National Cancer Institute listed in the Resources section of this report. This disease entry is based upon medical information available through August 1990. 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 Paget's Disease of the Breast, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The Skin Cancer Foundation 475 Park Avenue, South New York, NY 10001 212-725-5176 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 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 PAGET'S DISEASE OF THE BREAST, J.A. Sanchez, et al.; Am Fam Physician (August, 1987, issue 36 (2)). Pp. 145-147. MAMMOGRAPHY IN THE SYMPTOMATIC WOMAN. S. Edeiken, Cancer, (April, 1989, issue 1; 63 (7)). Pp. 1412-1414. RADIOTHERAPY FOR PAGET'S DISEASE OF THE NIPPLE: A CONSERVATIVE ALTERNATIVE. A.D. Stockdale, et al.; (September, 1989, issue 16;2 (8664)). Pp. 664-666. THE HISTOGENESIS OF MAMMARY AND EXTRAMAMMARY PAGET'S DISEASE, R.R. Jones, et al,; Histopathology (April, 1989, issue 14 (4)). Pp. 409-416.