Paget's disease of the breast
Encyclopedia
Paget's disease of the breast
(also known as Paget's disease of the nipple
) is a malignant condition that outwardly may have the appearance of eczema
, with skin changes involving the nipple of the breast.
The condition occurs when Paget's cells, which are large and irregular, form in the skin of the nipple. Although Paget believed the cells were not cancerous, it was later proved that the cells were themselves malignant, in addition to indicating another underlying breast cancer
. Since the condition in itself is often innocuous and limited to a surface appearance, it is sometimes dismissed, although actually indicative of a very serious underlying condition, breast cancer.
Extramammary Paget's disease
(EMPD) has the same histologic features as Paget's disease of the breast but different locations.
is made.
Paget's disease's symptoms may vary based on the stage of the disease. However, the main symptoms that can occur in Paget's disease include flaky or scaly skin
on the nipple, straw-colored or bloody nipple discharge, skin and nipple changes in only one breast or the flattened nipples. Patients may also experience crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both and fluctuating skin changes early on, making it appear as if the skin is healing
on its own. Some patients complain of burning sensations on the nipples or breasts. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to consult. Lumps or masses in the breast occur in 50% of the patients. In more advanced stages, the disease may cause tingling, increased sensitivity
and pain.
The first symptom is usually an eczema-like rash, usually only affecting one nipple. The skin of the nipple
and areola
may be red, itchy and inflamed. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area of cells. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.
The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to disappear for a while, which may make the patient think incorrectly that the condition has cleared up spontaneously.
Most women do not visit the doctor because they take Paget's disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed.
to confirm the diagnosis, and cytopathology
may also be helpful.
Paget's disease is difficult to diagnose due to its resemblance to dermatitis and eczema. Eczema tends to affect the areola first and then the nipple whereas paget's spreads from the nipple.
During a physical examination the doctor examines the unusual areas of the breast, especially the appearance of the skin on and around the nipples and feeling for any lumps or areas of thickening.
The most common test used to diagnose Paget's disease is the biopsy
, removal of a tissue sample from the affected area which is then examined under the microscope by a pathologist, who distinguishes Paget cells from other cell types by staining tissues to identify specific cells (immunohistochemistry
).
Samples of nipple discharge may also be examined under the microscope to determine whether Paget cells are present.
Imprint or scrape cytopathology
may be useful: scraping cells from the affected area, or pressing them onto a glass slide to be examined under the microscope.
or mastectomy
to surgically remove the tumour. Chemotherapy
and/or radiotherapy may be necessary.
Paget's disease is mainly treated with surgery
. However, the specific treatment often depends on the characteristics of the underlying breast cancer.
Invasive cancer or extensive ductal carcinoma in situ is primarily treated with modified radical mastectomies. The procedure consists in the removal of the breast, the lining over the chest muscles and a part of the lymph nodes from under the arm
. In cases of non-invasive cancers, simple mastectomies are performed in which only the breast with the lining over the chest muscles are removed.
Patients suffering from cancer that has not spread beyond the nipple and the surrounding area are often treated with breast-conserving surgery or lumpectomy
. They usually undergo radiation therapy
after the actual procedure to prevent recurrence. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer.
In most cases, adjuvant treatment is part of the treatment schema. This type of treatment is normally given to patients with cancer to prevent a potential recurrence of the disease. Whether adjuvant therapy is needed is considered upon the type of cancer and if the cancer cells have spread to the lymph nodes. In Paget's disease, the most common type of adjuvant therapy is radiation following breast-conservative surgery.
Adjuvant therapy may also consist of anticancer drugs or hormone therapies. Hormonal therapy reduces the production of hormones within the body, or prevent the hormones from stimulating the cancer cells to grow and it is commonly used in cases of invasive cancer by the mean of drugs such as tamoxifen
and anastrozole
.
Research into treatments for Paget's disease of the breast is ongoing. Cancer doctors use clinical trials to assess new treatments.
Furthermore, patients with an identifiable associated underlying breast tumor have a survival rate of 38-40% at 5 years and a survival rate of 22-33% at 10 years. The death rate of metastatic breast carcinoma in patients with mammary Paget's disease and underlying cancer is 61.3%, with a 10-year cumulative survival rate of 33%.
, an English surgeon
who first described it in 1875. Several other diseases
are also named after Paget.
Breast
The breast is the upper ventral region of the torso of a primate, in left and right sides, which in a female contains the mammary gland that secretes milk used to feed infants.Both men and women develop breasts from the same embryological tissues...
(also known as Paget's disease of the nipple
Nipple
In its most general form, a nipple is a structure from which a fluid emanates. More specifically, it is the projection on the breasts or udder of a mammal by which breast milk is delivered to a mother's young. In this sense, it is often called a teat, especially when referring to non-humans, and...
) is a malignant condition that outwardly may have the appearance of eczema
Eczema
Eczema is a form of dermatitis, or inflammation of the epidermis . In England, an estimated 5.7 million or about one in every nine people have been diagnosed with the disease by a clinician at some point in their lives.The term eczema is broadly applied to a range of persistent skin conditions...
, with skin changes involving the nipple of the breast.
The condition occurs when Paget's cells, which are large and irregular, form in the skin of the nipple. Although Paget believed the cells were not cancerous, it was later proved that the cells were themselves malignant, in addition to indicating another underlying breast cancer
Breast cancer
Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...
. Since the condition in itself is often innocuous and limited to a surface appearance, it is sometimes dismissed, although actually indicative of a very serious underlying condition, breast cancer.
Extramammary Paget's disease
Extramammary Paget's disease
Extramammary Paget’s disease , also Extramammary Paget disease, is a rare, slow-growing, usually non-invasive intraepithelial adenocarcinoma outside of the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis...
(EMPD) has the same histologic features as Paget's disease of the breast but different locations.
Symptoms
On average, a woman may experience signs and symptoms for six to eight months before a diagnosisDiagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
is made.
Paget's disease's symptoms may vary based on the stage of the disease. However, the main symptoms that can occur in Paget's disease include flaky or scaly skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
on the nipple, straw-colored or bloody nipple discharge, skin and nipple changes in only one breast or the flattened nipples. Patients may also experience crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both and fluctuating skin changes early on, making it appear as if the skin is healing
Healing
Physiological healing is the restoration of damaged living tissue, organs and biological system to normal function. It is the process by which the cells in the body regenerate and repair to reduce the size of a damaged or necrotic area....
on its own. Some patients complain of burning sensations on the nipples or breasts. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to consult. Lumps or masses in the breast occur in 50% of the patients. In more advanced stages, the disease may cause tingling, increased sensitivity
Sensitivity (human)
The sensitivity or insensitivity of a human, often considered with regard to a particular kind of stimulus, is the strength of the feeling it results in, in comparison with the strength of the stimulus...
and pain.
The first symptom is usually an eczema-like rash, usually only affecting one nipple. The skin of the nipple
Nipple
In its most general form, a nipple is a structure from which a fluid emanates. More specifically, it is the projection on the breasts or udder of a mammal by which breast milk is delivered to a mother's young. In this sense, it is often called a teat, especially when referring to non-humans, and...
and areola
Areola
This article is about the breast tissue. For the entomology term, see the glossary of Lepidopteran terms. For an artistic cloud motif, see aureola. For the cactus feature, see Areole....
may be red, itchy and inflamed. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area of cells. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.
The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to disappear for a while, which may make the patient think incorrectly that the condition has cleared up spontaneously.
Most women do not visit the doctor because they take Paget's disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed.
Diagnosis
Recommended tests are a mammogram and a biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
to confirm the diagnosis, and cytopathology
Cytopathology
Cytopathology is a branch of pathology that studies and diagnoses diseases on the cellular level. The discipline was founded by Rudolf Virchow in 1858. A common application of cytopathology is the Pap smear, used as a screening tool, to detect precancerous cervical lesions and prevent cervical...
may also be helpful.
Paget's disease is difficult to diagnose due to its resemblance to dermatitis and eczema. Eczema tends to affect the areola first and then the nipple whereas paget's spreads from the nipple.
During a physical examination the doctor examines the unusual areas of the breast, especially the appearance of the skin on and around the nipples and feeling for any lumps or areas of thickening.
The most common test used to diagnose Paget's disease is the biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
, removal of a tissue sample from the affected area which is then examined under the microscope by a pathologist, who distinguishes Paget cells from other cell types by staining tissues to identify specific cells (immunohistochemistry
Immunohistochemistry
Immunohistochemistry or IHC refers to the process of detecting antigens in cells of a tissue section by exploiting the principle of antibodies binding specifically to antigens in biological tissues. IHC takes its name from the roots "immuno," in reference to antibodies used in the procedure, and...
).
Samples of nipple discharge may also be examined under the microscope to determine whether Paget cells are present.
Imprint or scrape cytopathology
Cytopathology
Cytopathology is a branch of pathology that studies and diagnoses diseases on the cellular level. The discipline was founded by Rudolf Virchow in 1858. A common application of cytopathology is the Pap smear, used as a screening tool, to detect precancerous cervical lesions and prevent cervical...
may be useful: scraping cells from the affected area, or pressing them onto a glass slide to be examined under the microscope.
Treatment
Paget's disease of the breast is a type of cancer of the breast. Treatment usually involves a lumpectomyLumpectomy
Lumpectomy is a common surgical procedure designed to remove a discrete lump, usually a benign tumor or breast cancer, from an affected man or woman's breast...
or mastectomy
Mastectomy
Mastectomy is the medical term for the surgical removal of one or both breasts, partially or completely. Mastectomy is usually done to treat breast cancer; in some cases, women and some men believed to be at high risk of breast cancer have the operation prophylactically, that is, to prevent cancer...
to surgically remove the tumour. Chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
and/or radiotherapy may be necessary.
Paget's disease is mainly treated with surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
. However, the specific treatment often depends on the characteristics of the underlying breast cancer.
Invasive cancer or extensive ductal carcinoma in situ is primarily treated with modified radical mastectomies. The procedure consists in the removal of the breast, the lining over the chest muscles and a part of the lymph nodes from under the arm
Arm
In human anatomy, the arm is the part of the upper limb between the shoulder and the elbow joints. In other animals, the term arm can also be used for analogous structures, such as one of the paired forelimbs of a four-legged animal or the arms of cephalopods...
. In cases of non-invasive cancers, simple mastectomies are performed in which only the breast with the lining over the chest muscles are removed.
Patients suffering from cancer that has not spread beyond the nipple and the surrounding area are often treated with breast-conserving surgery or lumpectomy
Lumpectomy
Lumpectomy is a common surgical procedure designed to remove a discrete lump, usually a benign tumor or breast cancer, from an affected man or woman's breast...
. They usually undergo radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
after the actual procedure to prevent recurrence. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer.
In most cases, adjuvant treatment is part of the treatment schema. This type of treatment is normally given to patients with cancer to prevent a potential recurrence of the disease. Whether adjuvant therapy is needed is considered upon the type of cancer and if the cancer cells have spread to the lymph nodes. In Paget's disease, the most common type of adjuvant therapy is radiation following breast-conservative surgery.
Adjuvant therapy may also consist of anticancer drugs or hormone therapies. Hormonal therapy reduces the production of hormones within the body, or prevent the hormones from stimulating the cancer cells to grow and it is commonly used in cases of invasive cancer by the mean of drugs such as tamoxifen
Tamoxifen
Tamoxifen is an antagonist of the estrogen receptor in breast tissue via its active metabolite, hydroxytamoxifen. In other tissues such as the endometrium, it behaves as an agonist, hence tamoxifen may be characterized as a mixed agonist/antagonist...
and anastrozole
Anastrozole
Anastrozole is an aromatase-inhibiting drug approved for treatment of breast cancer after surgery, as well as for metastasis in both pre and post-menopausal women. The severity of breast cancer is increased by estrogen, as sex hormones cause hyperplasia, and differentiation at estrogen receptor...
.
Research into treatments for Paget's disease of the breast is ongoing. Cancer doctors use clinical trials to assess new treatments.
Prognosis
If lymph nodes are negative, the five year and ten year survival is 85% and 79% respectively. If the lymph nodes are positive, it is 32% and 28%. Involvement of the lymph nodes do not directly cause any harm but it is a merely an indicator of systemic spread.Furthermore, patients with an identifiable associated underlying breast tumor have a survival rate of 38-40% at 5 years and a survival rate of 22-33% at 10 years. The death rate of metastatic breast carcinoma in patients with mammary Paget's disease and underlying cancer is 61.3%, with a 10-year cumulative survival rate of 33%.
Epidemiology
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.History
The condition is named after Sir James PagetJames Paget
Sir James Paget, 1st Baronet was a British surgeon and pathologist who is best remembered for Paget's disease and who is considered, together with Rudolf Virchow, as one of the founders of scientific medical pathology. His famous works included Lectures on Tumours and Lectures on Surgical Pathology...
, an English surgeon
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
who first described it in 1875. Several other diseases
Paget's disease
Sir James Paget, a surgeon and pathologist, described several diseases, including:* Paget's disease of bone * Paget's disease of the breast* Paget-Schroetter disease* Extramammary Paget's disease...
are also named after Paget.