Paget's disease of the breast is a type of cancer that outwardly may have the appearance of eczema , with skin changes involving the nipple of the breast. The condition is an uncommon disease accounting for 1 to 4. The condition in itself often appears innocuous, limited to a surface appearance and it is sometimes dismissed, although actually indicative of underlying breast cancer. Paget's disease of the breast can affect the nipple and areola. Symptoms typically only affect one breast.
Breast pagets many years, mastectomywith or without the removal of lymph nodes under the arm Braest the same side of chest known as axillary lymph node dissectionwas regarded as the standard surgery for Paget disease of the breast 34. Blanchard; Michael G. According to the National Cancer Institute, the average age of diagnosis in women is 62, and Beeast men, The malignant changes occur without warning and for no known reason spontaneously. Limit postmenopausal hormone therapy. The exact cause of Paget's disease of the breast is not fully understood. There are several types of nipple biopsy, including the procedures described below.
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Resources for News Media. Talk with other breast cancer survivors. Core needle biopsy A core needle biopsy uses a long, hollow tube to extract a sample of tissue. Breasy a biopsy, a sample of the abnormal tissue will be taken using a needle. Paget's disease of the breast starts on the nipple and extends to pagetss dark circle of skin areola around the nipple. These cells are found in the epidermis Breast pagets layer of the skin of the nipple and the areola. The Breast pagets is an uncommon disease accounting for 1 to 4. The symptoms of Paget disease of the breast are often mistaken for those of some benign skin conditions, such as dermatitis or eczema 1 — 3. If you suspect you have Paget's disease of the breast, your initial appointment might be with your family doctor. The average age at diagnosis is 57 years, but the disease has been found in adolescents and pagers people in Breast pagets late 80s 23. Legal Requirements. Paget cells often have a large, round appearance under a microscope; they may be found as single cells or as small groups of cells Stacy moore nude the epidermis. Cancer Currents Blog. Grant Closeout. Using Trusted Ppagets.
Breast and nipple changes can be a sign of breast cancer.
- This website translates English to other languages using an automated tool.
- Paget's disease of the breast is a type of cancer that outwardly may have the appearance of eczema , with skin changes involving the nipple of the breast.
- During a mammogram, you stand in front of an X-ray machine designed for mammography.
- Paget's disease of the nipple is a rare form of breast cancer in which cancer cells collect in or around the nipple.
- Paget disease of the breast also known as Paget disease of the nipple and mammary Paget disease is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, which is called the areola.
- Breast and nipple changes can be a sign of breast cancer.
NORD gratefully acknowledges V. Paget's disease of the breast is a rare form of breast cancer that almost exclusively occurs in women. However, rare cases have been reported in men. Paget's disease of the breast is characterized by inflammatory, "eczema-like" changes of the nipple that may extend to involve the areola, which is the circular, darkened pigmented region of skin surrounding the nipple.
In individuals with Paget's disease of the breast, distinctive tumor cells known as Paget cells are present within the outermost layer of skin epidermis of the nipple, when viewed under a microscope. Most women with this disorder have an underlying cancer malignancy affecting the milk ducts ductal carcinoma.
The milk ducts are the channels that carry milk secreted by lobes of the breast to the nipple. The exact cause of Paget's disease of the breast is not fully understood. Paget's disease of the breast was originally reported in by Sir James Paget, an English surgeon, who also described an unrelated skeletal condition known as Paget's disease of the bone.
It is essential to note that these disorders are distinct entities that are medically unrelated. Some affected individuals may also have abnormal discharge from the nipple. Eventually, pain and sensitivity of the affected area may be present. As a result, diagnosis may be delayed, often up to six months or more. Most individuals with the condition eventually seek medical attention due to associated itching or burning sensations, soreness, or pain of the affected area.
Approximately 50 percent or more of affected individuals may have a lump or mass that may be felt palpated below the nipple. For example, in some instances, the nipple may turn inward retracted nipple. The overall disease course may vary greatly from one person to another, depending upon the nature and size of an underlying malignancy, whether a palpable breast tumor is present upon diagnosis, whether metastatic disease is present, specific treatment measures followed, and other possible factors.
Supportive evidence includes laboratory studies demonstrating that Paget cells and the underlying breast cancer share certain characteristics, thus appearing to originate from the same cell population. In addition, malignant cells have been shown to extend along breast ductal tissue from the underlying breast tumor to the nipple. Accordingly, many researchers have concluded that Paget cells appear to be derived from the lining epithelium of the milk ducts, invading and multiplying proliferating within the surface tissue epidermis of the nipple and adjacent areas of the areola.
The second theory suggests that the presence and spread of Paget cells represents a distinct disease process originating in the outer skin layer of the nipple. The malignant changes occur without warning and for no known reason spontaneously. This theory would explain the rare cases where no underlying malignancy is present or where the underlying malignancy is a different tumor type. Researchers speculate that genetic and immunologic abnormalities, environmental factors e.
Investigators at the National Cancer Institute and elsewhere are conducting ongoing basic research to learn more about the many factors that may result in cancer. In individuals with cancer, including breast malignancies, tumor development results from abnormal changes in the structure of certain cells.
The specific cause of such changes is unknown. In many patients, these abnormal changes may appear to occur spontaneously for unknown reasons sporadically. Cells that undergo malignant transformation typically revert to a less specialized, more primitive form anaplasia or loss of differentiation , meaning that they are no longer capable of performing their intended, specialized functions within the tissue in question.
This uncontrolled cellular growth may eventually result in invasion of surrounding tissues, infiltration of regional lymph nodes, and spread of the malignancy metastasis via the bloodstream, the lymphatic circulation, or other means.
However, there have been rare cases in which the disorder has occurred in men. It is thought to represent less than 5 percent of all breast cancer clinical presentations. Comparisons may be useful for a differential diagnosis:.
The term extramammary refers to regions other than or outside of the breasts. EMPD often involves the female or male external genitals e. EMPD of the vulva is associated with an underlying cancer carcinoma in about 30 percent of patients; involvement of the perianal area is associated with a distant or underlying carcinoma in up to approximately 73 percent of individuals.
Eczema is a common inflammatory condition characterized by itching pruritus and redness erythema of upper skin layers i. The condition is also often associated with blistering or scabbing, scaling, oozing, crusting, and thickening of affected skin areas. Bowen disease is a rare skin disorder. Affected individuals develop a slow-growing, reddish scaly patch or plaque on the skin.
Sun exposed areas of the skin are most often affected. Bowen disease only affects the outermost layer of the skin epidermis. Lesions are usually not painful or may not be associated with any symptoms asymptomatic. In most cases, treatment is highly successful. Bowen disease is considered a pre-cancerous condition, although the risk of developing skin cancer is less than 10 percent. The disorder usually affects older adults.
The exact cause of Bowen disease is unknown, although there are identified risk factors such as chronic sun exposure. Malignant melanoma is a form of skin cancer that develops from the melanin cells of the upper layer of the skin epidermis or from similar cells that may be found in moles nevi.
In early stages most melanomas do not produce any specific symptoms. Later, they may appear as lesions that do not heal or as existing moles that show changes in size or color. This type of skin cancer eventually spreads to the lower skin levels and adjacent tissue and may result in new tumor growths in vital organs of the body. The disorder can be confused with other skin conditions such as eczema potentially causing a delay in the diagnosis. During a biopsy a small sample of breast tissue is taken and examined under a microscope to determine whether Paget cells are present.
If a lump is found in the breast, a sample of that tissue will also be taken. A mammogram is an x-ray of the breast tissue and may be used to determine whether Paget cells are present.
Mammography is also used to detect or rule out whether an underlying cancer is present. Additional specialized imaging techniques such as an ultrasound or magnetic resonance imaging MRI may be used to create additional images of the breast and to determine whether an underlying cancer is present.
If nipple discharge is present, a sample may be taken and studied to detect the presence of Paget cells. If affected individuals have an underlying invasive cancer, then a sentinel lymph node biopsy may be performed. During this biopsy, a sample of tissue is taken from the lymph nodes under the armpits axillary lymph nodes to check and see whether cancer has spread to this area.
A physician will locate and remove the sentinel lymph node, which refers to the first or first few lymph node s to which cancer cells are most likely to spread. Decisions concerning the use of particular interventions should be made by physicians and other members of the health care team in careful consultation with the patient, based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks; patient preference, and other appropriate factors.
Treatment has traditionally included surgical removal of breast tissue, adjacent lymph nodes, and, in some cases, underlying muscles of the upper chest wall and other tissues modified radical or radical mastectomy. Some individuals may only require a simple mastectomy, in which the breast and the lining over the chest muscles are removed. In selected cases where there is no palpable breast mass and negative mammograms, or in those with disease confined to the nipple and areola, conservative tissue-sparing surgery lumpectomy may be recommended.
During this surgery, the nipple and areola are removed along with a small section of breast tissue. A physician will attempt to remove as little breast tissue as necessary.
Individuals who received a lumpectomy will receive follow up radiation therapy. Adjuvant therapy may include radiation therapy, anti-cancer drugs chemotherapy and hormone therapy. The underlying ductal cancers often have poor prognostic features than typical, often screen detected breast cancers including higher grade, larger size, and negative hormone receptor expression loss of estrogen and progesterone receptors.
During photodynamic therapy, the drug is administered to an affected individual and absorbed by the affected cells. A specific wavelength of light is used to active the drug which binds with oxygen creating a chemical that destroys the affected cells. Information on current clinical trials is posted on the Internet at www. All studies receiving U. For information about clinical trials sponsored by private sources, contact: www.
The effect of Paget disease on axillary lymph node metastases and survival in invasive ductal carcinoma. Fouad D. J Med Case Reports. BMC Dermatol. Am J Surg. Breast Cancer Res Treat.
Int Semin Surg Oncol. Marcus E. Curr Treat Options Oncol. Cancer Treat Rev. J Am Acad Dermatol. J Am Coll Surg. Kao GF. Mammary Paget Disease. Last Update May 12, Mayo Clinic for Medical Education and Research. Last Update March 29, National Cancer Institute. Last Update April 10, The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.
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About News Events Contact. General Discussion Summary Paget's disease of the breast is a rare form of breast cancer that almost exclusively occurs in women.
Most women with Paget's disease of the nipple also have either ductal carcinoma in situ DCIS or invasive breast cancer elsewhere in the breast. If you suspect you have Paget's disease of the breast, your initial appointment might be with your family doctor. Translated from English by This website translates English to other languages using an automated tool. If a patient notices any of the following symptoms, they should make an appointment with their physician immediately: Itching or tingling of the nipple or areola Redness Flaking, crusty, or thickened skin on or around the nipple A flattened nipple Discharge from the nipple that may be yellowish or bloody A lump in the breast Causes and Risk Factors The cause of Paget's disease of the breast is unknown, although there does appear to be a link between an underlying ductal cancer and the disease. What Is Cancer?
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The presence of three factors for the prognosis has been suggested, whether there is a palpable mass of the disease, whether lymph nodes are positive and whether there is an underlying malignant cancer. The death rate of metastatic breast carcinoma in patients with mammary Paget's disease and underlying cancer is Most patients diagnosed with Paget's disease of the nipple are over age 50, but rare cases have been diagnosed in patients in their 20s.
The average age at diagnosis is 62 for women and 69 for men. The disease is rare among both women and men. The condition is named after Sir James Paget , an English surgeon who first described the condition in . From Wikipedia, the free encyclopedia. For other diseases named after Paget, see Paget's disease disambiguation.
Breast Cancer Res. Journal of Carcinogenesis. On the connection between disease of the nipple and areola and tumors of the breast. Trans Pathol Soc Lond ; Archived from the original on Retrieved Robbins basic pathology 8th ed. Blanchard; Michael G. Sarr; David R. Farley Langenbeck's Archives of Surgery. Can Fam Physician. CS1 maint: archived copy as title link National Cancer Institute. Glandular and epithelial neoplasms ICD-O Small cell carcinoma Combined small cell carcinoma Verrucous carcinoma Squamous cell carcinoma Basal cell carcinoma Transitional cell carcinoma Inverted papilloma.
Renal cell carcinoma Endometrioid tumor Renal oncocytoma. Lobular carcinoma in situ Invasive lobular carcinoma. Medullary carcinoma of the breast Medullary thyroid cancer. Acinic cell carcinoma. Warthin's tumor Thymoma Bartholin gland carcinoma. See also Template:Epithelium and epithelial tissue. Fibroadenoma Phyllodes tumor. Medullary carcinoma. Male breast cancer Inflammatory breast cancer.
Atypical ductal hyperplasia. Breast cancer classification. Nipple adenoma. Categories : Breast cancer Epidermal nevi, neoplasms, cysts. Hidden categories: All articles with dead external links Articles with dead external links from March Articles with permanently dead external links CS1 maint: archived copy as title. Namespaces Article Talk. Views Read Edit View history. Most people with Paget disease of the breast also have one or more tumors inside the same breast.
These breast tumors are either ductal carcinoma in situ or invasive breast cancer 1 — 3. Paget disease of the breast is named after the 19th century British doctor Sir James Paget, who, in , noted a relationship between changes in the nipple and breast cancer. Several other diseases are named after Sir James Paget, including Paget disease of bone and extramammary Paget disease, which includes Paget disease of the vulva and Paget disease of the penis.
These other diseases are not related to Paget disease of the breast. This fact sheet discusses only Paget disease of the breast. Malignant cells known as Paget cells are a telltale sign of Paget disease of the breast. These cells are found in the epidermis surface layer of the skin of the nipple and the areola. Paget cells often have a large, round appearance under a microscope; they may be found as single cells or as small groups of cells within the epidermis.
Paget disease of the breast occurs in both women and men, but most cases occur in women. Approximately 1 to 4 percent of all cases of breast cancer also involve Paget disease of the breast. The average age at diagnosis is 57 years, but the disease has been found in adolescents and in people in their late 80s 2 , 3.
Doctors do not fully understand what causes Paget disease of the breast. The most widely accepted theory is that cancer cells from a tumor inside the breast travel through the milk ducts to the nipple and areola. This would explain why Paget disease of the breast and tumors inside the same breast are almost always found together 1 , 3. A second theory is that cells in the nipple or areola become cancerous on their own 1 , 3. This would explain why a few people develop Paget disease of the breast without having a tumor inside the same breast.
Moreover, it may be possible for Paget disease of the breast and tumors inside the same breast to develop independently 1. The symptoms of Paget disease of the breast are often mistaken for those of some benign skin conditions, such as dermatitis or eczema 1 — 3.
These symptoms may include the following:. Because the early symptoms of Paget disease of the breast may suggest a benign skin condition, and because the disease is rare, it may be misdiagnosed at first. People with Paget disease of the breast have often had symptoms for several months before being correctly diagnosed. A nipple biopsy allows doctors to correctly diagnose Paget disease of the breast. There are several types of nipple biopsy, including the procedures described below.
In some cases, doctors may remove the entire nipple 1. A pathologist then examines the cells or tissue under a microscope to look for Paget cells. Most people who have Paget disease of the breast also have one or more tumors inside the same breast.
In addition to ordering a nipple biopsy, the doctor should perform a clinical breast exam to check for lumps or other breast changes. As many as 50 percent of people who have Paget disease of the breast have a breast lump that can be felt in a clinical breast exam. The doctor may order additional diagnostic tests, such as a diagnostic mammogram , an ultrasound exam, or a magnetic resonance imaging scan to look for possible tumors 1 , 2. For many years, mastectomy , with or without the removal of lymph nodes under the arm on the same side of chest known as axillary lymph node dissection , was regarded as the standard surgery for Paget disease of the breast 3 , 4.
This type of surgery was done because patients with Paget disease of the breast were almost always found to have one or more tumors inside the same breast. Even if only one tumor was present, that tumor could be located several centimeters away from the nipple and areola and would not be removed by surgery on the nipple and areola alone 1 , 3 , 4.
Studies have shown, however, that breast-conserving surgery that includes removal of the nipple and areola, followed by whole-breast radiation therapy, is a safe option for people with Paget disease of the breast who do not have a palpable lump in their breast and whose mammograms do not reveal a tumor 3 — 5. People with Paget disease of the breast who have a breast tumor and are having a mastectomy should be offered sentinel lymph node biopsy to see whether the cancer has spread to the axillary lymph nodes.
If cancer cells are found in the sentinel lymph node s , more extensive axillary lymph node surgery may be needed 1 , 6 , 7. The prognosis, or outlook, for people with Paget disease of the breast depends on a variety of factors, including the following:.
The presence of invasive cancer in the affected breast and the spread of cancer to nearby lymph nodes are associated with reduced survival. This compares with a 5-year relative survival of For women with both Paget disease of the breast and invasive cancer in the same breast, the 5-year relative survival declined with increasing stage of the cancer stage I, However, people who have Paget disease of the breast may be eligible to enroll in clinical trials to evaluate new treatments for breast cancer in general, new ways of using existing breast cancer treatments, or strategies for preventing breast cancer recurrence.
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Paget's Disease of the Nipple: Symptoms, Diagnosis, and More
Back to Health A to Z. It causes eczema-like changes to the skin of the nipple and the area of darker skin surrounding the nipple areola. Paget's disease of the nipple always starts in the nipple and may extend to the areola. The affected skin is often sore and inflamed, and it can be itchy or cause a burning sensation. The nipple can sometimes be ulcerated. If you scratch it, or if it's left untreated, it can bleed, become ulcerated or scab over. However, you should still have it checked by a doctor.
In 9 out of 10 cases this is an invasive breast cancer. Invasive cancer is where cancerous cells invade the surrounding breast tissue. Some women with Paget's disease have invasive breast cancer but do not have a lump.
This is where the cancerous cells are contained in 1 or more areas of the breast and have not spread. Some women appear to be more at risk of developing breast cancer, but not specifically Paget's disease.
There are no known causes that lead to a specific type of breast cancer. Read more about the risk factors for breast cancer. As Paget's disease of the nipple is associated with breast cancer, the sooner it's diagnosed, the better the outcome is likely to be.
Also see your GP if you develop a lump in your breast. Although most breast lumps are not cancerous, it's important to have them checked out. A small tissue sample punch biopsy of the skin will be taken from your nipple and examined under a microscope to see if it's cancerous.
If the results of the biopsy indicate Paget's disease, you'll have a mammogram a low-dose breast X-ray to investigate further. Read more about diagnosing Paget's disease of the nipple. Surgery is the main treatment for Paget's disease of the nipple. You may also need further treatment if you have invasive breast cancer.
This may be a combination of:. If Paget's disease is detected and treated in its early stages, there's a good chance of a full recovery. Read more about treating Paget's disease of the nipple. In some areas, women aged 47 to 49 and 71 to 73 are also invited for screening as part of a study looking at whether the age range for breast screening should be extended. Read more about reducing the risks of breast cancer. Page last reviewed: 22 October Next review due: 22 October Symptoms of Paget's disease of the nipple Paget's disease of the nipple always starts in the nipple and may extend to the areola.