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Paget's Disease of the Breast


Disease: Paget's Disease of the Breast Paget's Disease of the Breast
Category: Tumors

Disease Definition:

This is a rare form of breast cancer which makes 5% of all breast cancer cases. In Paget's disease of the breast, the condition starts with affecting the breast ducts and extends to the skin of the nipple and to areola. Paget's disease of the breast has nothing to do with Paget's disease of the bone, which is a disease affecting the metabolism of the bones.


Women over the age of 50 are statistically more prone to Paget's disease of the breast. An underlying infiltrating ductal breast cancer is common in women with Paget's disease of the breast. The nipple may be the sole affected part in rare cases.

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Symptoms, Causes


As Paget's disease of the breast affects the nipple and the areola, it is easy to confuse it with the symptoms of skin irritation (dermatitis) or another noncancerous (benign) skin condition of the nipple. Some of the signs and symptoms of this disease may include:


  • Itching
  • Redness
  • A burning sensation
  • A flattened or inverted nipple
  • Flaky or scaly skin on the nipple
  • Straw-colored or bloody nipple discharge
  • Affected areas beyond the nipple and areola
  • A distinct lump underneath the nipple and areola
  • Skin and nipple changes usually in one breast only
  • Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
  • Fluctuating skin changes early on, making it appear as if the skin is healing on its own


Six to eight months may pass on the symptoms until a diagnosis is made. A woman should check her nipples and areolas during regular self exams of her breasts. In the case of sensing a lump or skin irritation for more than a month, a physician should be consulted. A woman should make a follow-up appointment with her physician in case she is receiving treatment for a skin lesion on her breast, and the condition is persisting despite treatment. She might need a biopsy for further evaluation of the affected area.


The cause of Paget's disease of the breast is yet unknown. But there is pressing evidence that the disease is caused by an underlying ductal or invasive breast cancer. Nipples and areolas are infected with cancer cells that travel through milk ducts after being separated from the original tumor.


There is another theory that suggests the spontaneous transform of the healthy skin cells on the nipples to cancerous cells. No underlying breast cancer can be found in rare cases of Paget's disease of the breast. The risk factors of developing Paget's disease of the breast are the same as the risk factors that are involved in the development of other types of breast cancer. The factors that increase the likelihood of developing breast cancer are:



As a woman grows old, her risk of developing breast cancer increases.


A personal history of breast cancer:

If a woman had breast cancer in one of her breasts, she has an increased chance of developing cancer in the other one.


Family history:

In case a woman has a close female relative with breast cancer or ovarian cancer or both, or even a male relative with breast cancer, she is at higher risk of developing breast cancer.


Genetic predisposition:

BRCA1 or BRCA2 among other genes, if defected, increase the risk of developing breast cancer, as is the case with ovarian and colon cancers.


Radiation exposure:

A woman is at greater risk of developing breast cancer if she has previously had radiation treatment in the chest region.


Excess weight:

Being overweight in according to one's height or age, may make her more susceptible to breast cancer, especially if the extra weight is gained after menopause. Excessive weight gain during puberty is also one of the factors that increase the risk of developing breast cancer later on.


Exposure to estrogen:

The longer the duration is from the onset of menstruation to menopause, the greater are the risks of developing breast cancer, for estrogen is at higher levels during this period. This might be the case with early menstruation before the age of 12 or late menopause after the age of 55. Some women, who take estrogen for hormone replacement therapy, are at higher risks of developing breast cancer.



Statistics show that white women are more likely to have breast cancer than black or Hispanic women, but death form the disease is more common in black women.


The presence of one or more of the above mentioned factors does not necessarily lead to breast cancer, for most of the diagnosed women do not have any known risk factors.





Surgery is required for most of the cases of Paget's disease. The surgery type is dictated by the condition of the skin around the nipple and the stage of the underlying cancer. Surgical options include:


Simple mastectomy:

In this type of surgery the entire breast is surgically removed, except for the axillary lymph nodes. When an underlying noninvasive breast cancer exists but hasn't affected the lymph nodes yet, simple mastectomy may be recommended.



This is the procedure in which only the affected parts of the breast are surgically removed. The procedure calls for the removal of the nipple and the areola along with a cone-shaped section from the breast. The surgeon should be careful not to remove more than what is needed, for the removed cone should have a thin layer of healthy cells to ensure that no cancerous cells are left back. As a form of adjuvant treatment, radiation therapy is needed after lumpectomy. In case a woman should not be subjected to radiation, lumpectomy is not recommended. After treatment, the woman can have a nipple reconstruction.



In case of diagnosing an invasive breast cancer, axillary lymph nodes should be constantly checked to ensure that the cancer has not spread to them. Sentinel lymph node biopsy is the procedure carried out in the examination.


The sentinel node is located by a surgeon during biopsy. The sentinel node is the first lymph node that gets invaded by breast cancer cells. In case the sentinel node is not affected, it is highly unlikely to find other lymph nodes that are infected by cancer cells; hence no further nodes need be removed. But the removal of other nodes might be needed, if the sentinel node has turned a positive result in the test.



The recurrence of the cancer may be prevented after surgery by undergoing adjuvant therapy which includes anti-cancer drugs or hormone therapy. The course of this therapy depends on the extent of the cancer or the results of the tumor test, whether or not it has positive results for certain characteristics like having estrogen or progesterone receptors.


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