Paget Disease of the Breast

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• Paget disease of the breast is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, known as the areola.
• Most of the time, people with Paget disease of the breast also have one or more tumors inside the same breast.
• Paget disease of the breast may be misdiagnosed at first because its early symptoms are similar to those caused by some benign skin conditions.
• The outlook for people diagnosed with Paget disease of the breast depends on a variety of factors, including the presence or absence of invasive cancer in the affected breast and, if invasive cancer is present, whether or not it has spread to nearby lymph nodes.

Paget disease of the breast (also known as Paget disease of the nipple and mammary Paget disease) is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, which is called the areola. Most people with Paget disease of the breast also have one or more tumors inside the same breast. These breast tumors are either ductal carcinoma in situ or invasive breast cancer.

Paget disease of the breast is named after the 19th century British doctor Sir James Paget, who, in 1874, noted a relationship between changes in the nipple and breast cancer. (Several other diseases are named after Sir James Paget, including Paget disease of bone and extramammary Paget disease, which includes Paget disease of the vulva and Paget disease of the penis. These other diseases are not related to Paget disease of the breast. This fact sheet discusses only Paget disease of the breast.)

Malignant cells known as Paget cells are a telltale sign of Paget disease of the breast. These cells are found in the epidermis (surface layer) of the skin of the nipple and the areola. Paget cells often have a large, round appearance under a microscope; they may be found as single cells or as small groups of cells within the epidermis.