Paget's Disease of the Breast

Carolyn Vachani RN, MSN, AOCN
The Abramson Cancer Center of the University of Pennsylvania
Last Modified: April 23, 2009

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Question
I have been having itching, burning, and redness on my left breast. The pain wakes my up at night. I did not feel any lumps in either breast. I was reading a magazine about breast cancer, and came across Paget's disease. I just had a mammogram, but I don't know the result yet. Would mammogram be able to detect Paget's disease? What is Paget's disease anyway?  
Thanks
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Answer
Carolyn Vachani, RN, MSN, AOCN, OncoLink's Nurse Educator, responds:

The term Paget's disease was named after Sir James Paget who first reported the association between skin changes of the nipple and the subsequent development of breast cancer in 1874. While it may be sometimes called Paget’s Disease (PD) of the breast or Mammary PD, it is almost always involving the nipple. Between 1-4% of all primary breast cancers present with PD. Though PD is not a cancer itself, the majority of women who present with PD of the nipple or breast have an underlying cancer. Physicians theorize that the nipple changes are a result of the cancer cells spreading through the ducts in the breast to the skin surface. PD is a cutaneous manifestation of an underlying cancer.

Most patients present with a rash, redness and inflammation of the nipple and areolar area (dark skin around the nipple). There is often bleeding, weeping, itching, burning sensation and ulceration of the area. About half of women will also have a lump detected elsewhere in the breast. Of those with no detectable lump, 63% will have a negative mammogram. To accurately diagnose PD, a biopsy of the affected area is necessary. The work up for Paget's disease includes a history and physical exam, mammogram, biopsy, and routine blood tests.

Traditionally, this disease was treated with modified radical mastectomy. The rationale for this was that the nipple and areola would need to be removed and the possibility of involvement of other areas of the breast with tumor. In patients without a palpable mass and no evidence of tumor on mammography or disease confined to the nipple/areolar area, lumpectomy and removal of the nipple/areolar area, followed by radiation therapy to the whole breast is an acceptable alternative to mastectomy. The nipple/areolar can be reconstructed and this can result in good cosmetic results. Local recurrence rates and survival are similar to those seen with more common types of breast cancer when comparisons are made between similar stages (size of tumor and degree of spread).