Pagets Disease of Breast – Causes, Symptoms and Treatment

Paget's disease of the breast, also called mammary Paget's disease, is a rare breast condition that is often associated with underlying breast cancer. It is believed that Paget's disease of the breast occurs when invasive carcinoma or intraductal carcinoma (cancer of the milk ducts) spreads through the milk ducts to the nipple.
Although in most cases the underlying breast cancer is extensive, in 10% of the cases, cancer only affects the nipple and surrounding tissue. Rarely, there is no detectable underlying breast cancer.

Paget's disease of the breast, also called mammary Paget's disease, is a rare breast condition that is often associated with underlying breast cancer. It is believed that Paget's disease of the breast occurs when invasive carcinoma or intraductal carcinoma (cancer of the milk ducts) spreads through the milk ducts to the nipple.
Although in most cases the underlying breast cancer is extensive, in 10% of the cases, cancer only affects the nipple and surrounding tissue. Rarely, there is no detectable underlying breast cancer.

Paget's disease of the nipple, also called Paget's disease of the breast, is an uncommon type of cancer that forms in or around the nipple (1, 2, 3). More than 95 percent of people with Paget's disease of the nipple also have underlying breast cancer; however, Paget's disease of the nipple accounts for less than 5 percent of all breast cancer.

Causes
If invasive breast cancer or ductal carcinoma in situ (DCIS) is already present in the breast as a tumor, cells might drift off from the tumor and float up through the milk ducts, where they enter the nipple.

In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. Researchers suggest that in those cases, nipple skin cells may spontaneously change into cancer cells.
Signs and symptoms
Paget's disease of the breast affects your nipple and its surrounding skin (areola). It's easy to mistake the signs and symptoms for skin irritation (dermatitis) or another noncancerous (benign) skin condition of the nipple. In the early stages of the disease, you might notice only some flaky or scaly skin on your nipple, accompanied by slight itching or redness. These skin changes can come and go, making it appear as if your skin is healing on its own. On average, a woman may experience signs and symptoms for six to eight months before a diagnosis of Paget's disease of the breast is made.

Surgery
Whether you will need surgery and what type of surgery your doctor recommends will depend on the underlying cells. If there is a cancer diagnosis, the treatment will be based on the cancer diagnosis - not the Paget's. An early stage cancer will probably involve a lumpectomy, removing the lump and the breast tissue around it. A more advanced cancer may require a mastectomy, removal of the breast, and other treatments.

Skin-sparing mastectomy. In this procedure, the surgeon makes an incision around your nipple and areola, removes all of the underlying breast tissue, but preserves the overlying skin. Breast reconstruction with breast implants or with your body's own tissue (autologous reconstruction) is usually performed during the same surgery. Skin-sparing mastectomy isn't an option if the skin changes associated with Paget's disease have spread beyond your areola.
Chemotherapy/Hormonal Therapy
These adjuvant treatments may be prescribed by your health care team to treat the cancer that produced the Paget's symptoms. There are many different drug therapies available. These treatments may involve drugs such as tamoxifen, which are meant to prevent the cancer from growing again. You may need more aggressive treatments if the cancer has spread to your lymph nodes or other sites.
by peter hutch