What is Paget’s Disease of the Nipple?

Paget’s disease of the nipple is a rare presentation of breast cancer that appears as a rash on the nipple. The changes in the nipple usually indicate an underlying cancer in the breast ducts, often just under the nipple, which has extended onto the surface of the nipple. In many cases, this sign of breast cancer indicates a small ductal carcinoma in situ (DCIS), a very early breast cancer. At other times, Paget’s disease of the nipple may indicate an invasive (infiltrating) cancer elsewhere in the breast. In some instances, women who show signs of Paget’s disease of the nipple will also have an abnormal mammogram or present a breast lump upon physical examination.

Paget’s disease of the nipple should not be confused with Paget’s disease of the bone, a severe bone disease. Both conditions were first documented by Sir James Paget, a British surgeon and physiologist, but are completely unrelated diseases. In rare cases, Paget’s disease of the nipple can also affect men.

How is Paget’s Disease of the Nipple Detected?

Paget’s disease of the nipple is often first noticed when signs of the disease appear. Signs of Paget’s disease usually only occur on one nipple and may include:

·         persistent crustiness, scaliness, or redness of the nipple

·         itching or burning of the nipple and surrounding areola

·         bleeding or oozing from the nipple and areola

Paget’s disease often mimics skin conditions, such as breast eczema—a highly treatable condition characterized by recurring red, itchy patches or weeping blisters around the nipple. However, the signs of Paget’s disease do not typically disappear with routine treatment for eczema or infection and usually only affect one nipple.

If signs of Paget’s disease of the nipple are persistent, a mammogram is usually ordered to check for cancer in the breast itself. It is important to note that sometimes the underlying abnormal breast mass will be difficult to visualize on a mammogram. Therefore, a "negative" mammogram in the face of an abnormal nipple finding will still require further investigation.

Next, a biopsy of the nipple tissue will be performed (this is sometimes referred to as a "punch biopsy" and involves removing a small amount of tissue to confirm the presence of cancer). If the mammogram or other breast imaging exams reveal suspicious regions within the breast or if a breast lump is detected by physical exam, a biopsy of the abnormal area(s) within the breast will also be performed. A biopsy of the nipple tissue will usually be performed.

How is Paget’s Disease of the Nipple Treated?

Paget’s disease of the nipple is usually treated with surgery. If there is cancer in the breast, the location of the cancer will determine which type of surgery is preferable (lumpectomy or mastectomy). For example, if the cancer is non-invasive (i.e.,DCIS) and just under the nipple, a lumpectomy may be performed to remove the nipple and underlying cancer. Radiation therapy is usually given after lumpectomy. If the cancer is far from the nipple, a mastectomy may need to be performed to remove the affected nipple and the entire breast.  Chemotherapy or drug therapy (such as tamoxifen) may be necessary depending on the final results of surgery.

Paget’s disease typically has an excellent prognosis if it is confined to the nipple and underlying breast ducts. In this case, the axillary (armpit) lymph nodes are usually cancer-free. However, the survival rate can be lower if Paget’s disease of the nipple is associated with an invasive breast cancer or metastatic disease (the cancer has spread out of the breast to other areas of the body).

Recently, some physicians have advocated only removing the nipple and areola (sometimes followed by radiation therapy) in patients whose Paget’s disease is not associated with any identifiable underlying breast cancer. This allows the woman to maintain her breast. After proper treatment has been completed, an artificial nipple may be recreated using skin grafts and tattooing.

Updated: February 28, 2001

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