Breast Investigators

Source: BreastCancer.org

Paget's Disease of the Nipple

Paget's disease of the nipple is a rare form of breast cancer in which cancer cells collect in or around the nipple. The cancer usually affects the ducts of the nipple first (small milk-carrying tubes), then spreads to the nipple surface and the areola (the dark circle of skin around the nipple). The nipple and areola often become scaly, red, itchy, and irritated.

According to the National Cancer Institute, Paget's disease of the nipple accounts for less than 5% of all breast cancer cases in the United States. Being aware of the symptoms is important, given that more than 97% of people with Paget's disease also have cancer, either DCIS or invasive cancer, somewhere else in the breast. The unusual changes in the nipple and areola are often the first indication that breast cancer is present.

Doctors are not yet completely sure how Paget's disease develops. One possibility is that the cancer cells start growing inside the milk ducts within the breast and then make their way out to the nipple surface. This would appear to explain why so many people with Paget's disease of the nipple have a second area of cancer within the breast. Another theory is that the cells of the nipple itself become cancerous. This theory would explain the small number of people who: (1) only have Paget's disease in the nipple, or (2) have a second breast cancer that appears to be completely separate from the Paget's disease.

Paget's disease of the nipple is more common in women, but like other forms of breast cancer, it can also affect men. The disease usually develops after age 50. According to the National Cancer Institute, the average age of diagnosis in women is 62, and in men, 69.

Symptoms and Diagnosis of Paget’s Disease

Paget's disease causes the skin on and around the nipple to become red, sore, and flaky, or scaly. At first, these symptoms tend to come and go.

Over time, symptoms of Paget's disease usually worsen and may include:

• itching, tingling, and/or a burning sensation
• pain and sensitivity
• scaling and thickening of the skin
• flattening of the nipple
• yellowish or bloody discharge from the nipple

Because Paget's disease of the nipple is rare, doctors often mistake it for eczema (severe skin rash and inflammation), an infection or injury, or some other skin condition. For many people, it can take several months to get a correct diagnosis. If you have any of the above symptoms and they persist in spite of treatment, get them checked out by a breast specialist. In most cases, Paget's disease affects one breast, not both.

Diagnosing Paget's disease usually involves the following steps:

• A physical examination of the breasts, with special attention paid to the area around the nipple. Your doctor may be able to feel a lump or mass in the breast.
• A mammogram to check the nipple area and also to look for evidence of cancer in other areas of the breast.
• Ultrasound and/or breast MRI to create additional images of the breast and check for other areas of cancer.
• Biopsy of the nipple and areola. A breast surgeon will perform minor surgery to remove a small piece of tissue from the nipple and areola area and examine it under a microscope. If there is unusual discharge from the breast, your doctor will take a sample of that for examination as well.