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$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.