Paget's Disease of the Nipple

________________________________________________________________________

KEY POINTS

• Paget's disease is a growth of abnormal cells in the milk ducts and skin of the nipple. It is a rare form of breast cancer.
• Treatment may include medicine such as chemotherapy, hormone therapy, or biologic therapy. It may also include radiation therapy or surgery.
• Talk with your healthcare provider about ways to best care for yourself during treatment.

________________________________________________________________________

What is Paget's disease of the nipple?

Paget's disease is a growth of abnormal cells in the milk ducts and skin of the nipple. It is a rare form of breast cancer. It usually affects women and is rare in men.

The sooner cancer is found and treated, the better your chances for recovery. However, even advanced cancer can usually be treated. Treatment may slow or stop the growth of the cancer and ease symptoms for a time. Ask your healthcare provider what you can expect with the type of cancer that you have.

What is the cause?

The cause of Paget’s disease of the breast is not known. It may spread to the nipple from a tumor inside the breast, or the tumor may start in the milk ducts of the nipple.

What are the symptoms?

Symptoms may include:

• Bleeding, oozing, and crusting of your nipple and the circular, dark area around the nipple.
• Itching and burning of the nipple and skin around the nipple
• A sore on the nipple that does not heal
• Change in nipple shape or appearance
• Red, scaly skin on and around the nipple

Usually only 1 nipple is affected. Sometimes no changes in the skin can be seen. You may also have a lump in your breast, which you may or may not be able to feel.

It is very important to see a healthcare provider if you have these symptoms. The symptoms of Paget's disease of the nipple may look like a harmless skin irritation or infection, which can delay its diagnosis and treatment.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you. You may have tests such as:

• Breast biopsy, which is the removal of a small sample of tissue for testing. The tissue is examined for the presence of cancer.
• Mammogram, which is an X-ray of the breast to look for cancer or to check a lump felt in the breast.
• MRI, which uses a strong magnetic field and radio waves to show detailed pictures of the breast tissue
• Ultrasound, which uses sound waves to show pictures of the breast tissue

If you have discharge from a nipple, some of the discharge can be placed on a microscope slide and examined for cancer cells.

How is it treated?

You and your healthcare provider will discuss possible treatments. You may also talk with a surgeon, medical oncologist, and radiation oncologist. Oncologists are cancer specialists.

Some things to discuss with your healthcare provider when making treatment decisions are:

• Your other medical conditions
• The stage of the cancer (how advanced the cancer is)
• The effect of hormones on the cancer
• The type of breast cancer
• Whether the cancer has spread to lymph nodes or other parts of your body

Possible treatments are:

• Surgery, such as:
  • Surgery to remove just the nipple and surrounding tissue
  • Lumpectomy to remove a lump and the nipple
  • Mastectomy to remove all of the breast
• Radiation therapy, which uses high-energy X-rays to kill cancer cells
• Chemotherapy (anticancer drugs), which uses medicine to kill cancer cells
• Hormone therapy, which uses medicine to stop hormones in your body from helping tumors grow
• Biological therapy or targeted therapy, which uses medicine designed to help your immune system fight the cancer or block the growth of cancer cells

Often, more than 1 type of treatment is used.

If you are going to have surgery to remove your breast, talk to your healthcare provider about the options for breast reconstruction.

How can I take care of myself?

If you have been diagnosed with Paget’s disease of the nipple:

• Talk about your cancer and treatment options with your healthcare provider. Make sure you understand your choices.
• Follow the full course of treatment prescribed by your healthcare provider.
• Ask your healthcare provider:
  • How and when you will get your test results
  • How long it will take to recover
  • If there are activities you should avoid and when you can return to your normal activities
  • How to take care of yourself at home
  • What symptoms or problems you should watch for and what to do if you have them
• Make sure you know when you should come back for a checkup. Keep all appointments for provider visits or tests.

Other things that may help include:

• Eat a healthy diet and get regular exercise as recommended by your healthcare provider.
• Get plenty of rest.
• Keep a healthy weight. Women who gain 20 to 30 pounds or more after their teens are more likely to get breast cancer than those who don't gain this much weight. This is because fat cells make estrogen, which can help breast cancer grow. Men who are very overweight may also be at greater risk for breast cancer.
• Ask your provider if you need to avoid drinking alcohol. It may interfere with medicines you are taking. Alcohol can also make it harder for white blood cells to fight infections.
• Talk to your healthcare provider about hormone replacement therapy. The female hormones estrogen and progesterone may increase your risk of breast cancer.
• Try to reduce stress and take time for activities that you enjoy. It may help to talk with a counselor about your illness.
• Talk with your family and your healthcare providers about your concerns. Ask your healthcare provider any questions you have about the disease, treatments, side effects of the treatments, sexual activity, support groups, and anything else that concerns you.
• Tell your provider if your treatment causes discomfort, nausea, or other symptoms. Usually there are ways to help you be more comfortable.

After your treatment, it is still important to check your breast area for changes. This may help you discover any signs that the cancer has come back or a new cancer has started.

There are many support services for people with breast cancer. You can find the names of groups and agencies from your healthcare provider or through your local American Cancer Society office.

For more information, contact:

• American Cancer Society, Inc.
  800-227-2345
  http://www.cancer.org
• National Cancer Institute
  800-422-6237 (TTY: 800-332-8615)
  https://www.cancer.gov/