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Paget’s Disease of the Nipple


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Paget's disease is a rare cancer of the nipple and areola, the darker skin around the nipple. It affects both men and women.

The signs and symptoms of Paget's disease include irritation around the nipple, flaky or crusty skin, itching of the skin, and nipple discharge. Most patients with Paget's disease also have an underlying breast cancer within the breast. Therefore, if you develop these signs/symptoms, it is important to see your doctor for breast imaging and a possible biopsy of the skin.


The first line of treatment for Paget's disease is surgery. The type of surgery you need will depend on how much of your breast is affected. Your surgeon will remove the nipple and areola, as well as any other cancer within your breast. In some instances, breast-conserving surgery — which removes only diseased tissue and leaves healthy breast tissue intact — can be an option.

Breast-Conserving Surgery
Usually, this type of surgery involves removing the nipple and areola removal and a lumpectomy to remove the cancer from the underlying breast tissue. Surgeons remove the cancerous area and a small margin of healthy tissue around it to be sure no cancer cells are left behind. Your doctor will discuss your options and help you decide what's right for you.

If you have an invasive cancer in addition to Paget's disease, your doctor may recommend removing your entire breast, called a mastectomy, to make sure all cancer cells are removed. If you decide you want reconstructive surgery to restore your breast, you can do so as part of your initial surgery or wait until later to have surgery.

The type of mastectomy you have — total mastectomy or modified radical mastectomy — depends on how advanced the cancer is.

Total or Simple Mastectomy
In this surgery, your surgeon removes your entire breast, but leaves your underarm lymph nodes intact.

Modified Radical Mastectomy
With a modified radical mastectomy, your surgeon removes the nipple, areola and all of the breast tissue without removing the underlying muscle.

Sentinel Lymph Node Biopsy
Doctors use this minor surgery to determine if cancer has spread beyond a primary tumor into your lymphatic system. A sentinel lymph node is the first place cancer cells may move to when they begin to spread. During the biopsy, your doctor injects a special dye (tracer) to locate the nodes and remove them to look for cancer cells. If they're free of cancer, it means it's unlikely that cancer has spread and it's not necessary to remove more lymph nodes.

Sylvester offers a novel treatment that prevents taking more lymph nodes, even if the sentinel node is suspicious. Before surgery, an ultrasound scan of the armpit can highlight which lymph nodes are involved (they will appear enlarged or swollen). When chemotherapy is used before surgery (neoadjuvant chemotherapy), it can convert a previously positive lymph node to negative when biopsied, which means more lymph nodes don't need to be removed.

Radiation Therapy
If you have breast-conserving surgery, rather than a mastectomy, you will also need to have radiation therapy to destroy any cancer cells left behind after surgery. If Paget's disease is confined to your nipple and areola and no cancer cells were found in your lymph nodes, you won't need any additional treatment after radiation therapy.

Chemotherapy is cancer-fighting medicine that's administered intravenously or by a pill. Chemotherapy also might be recommended before surgery, called neoadjuvant chemotherapy, to help reduce the size of the tumor and improve surgical results. Often, more than one chemotherapy medicines are used at the same time or one after the other to destroy different kinds of cancer cells that come from the same breast cancer.

Locations: If you need intravenous (infusion) chemotherapy, you can have it at the Comprehensive Treatment Unit (CTU) at Sylvester’s main location in Miami, a 12,000-square-foot unit that includes 33 recliners and 11 private rooms. If you prefer, you may have your infusion treatments at the Kendall, Plantation, Hollywood, Coral Springs, Coral Gables and Deerfield Beach locations.

Cold Cap Therapy to Minimize Hair Loss
Sylvester offers FDA-cleared cold cap therapy to reduce hair loss during chemotherapy. This therapy works by cooling the scalp during treatment.

Targeted Therapy
Targeted therapies are treatments designed to attack the molecular changes that make the cancer cells grow and spread. The FDA has approved several targeted therapies.

Herceptin, a common type of targeted therapy, treats HER2-positive breast cancers. In these breast cancer, the cancer cells have a gene mutation that creates too much HER2 protein, which stimulates cancer cell growth. Herceptin targets and blocks the receptors, so the signals to grow and spread can't be delivered. Herceptin also marks the cancer cells so the immune system can destroy them.

Hormone Therapy
After you have surgery, and possibly radiation therapy, you may need to have one or more additional therapies, such as chemotherapy, targeted therapy or hormone therapy. Since most people with Paget's disease of the nipple also have invasive breast cancer, your treatment will be based on the type and stage of cancer you have.

Why Choose Sylvester Comprehensive Cancer Center?

Sylvester is an NCI-designated cancer center. The National Cancer Institute has recognized Sylvester for its outstanding work conducting research in its laboratories, treating patients in its clinics and hospitals, and reaching out to medically underserved communities with innovative prevention strategies.

Renowned Braman Family Breast Cancer Institute at Sylvester. We offer treatments customized to your individual needs at one of the nation's best breast cancer diagnosis and care programs.

Breast Imaging Center of Excellence designation by the American College of Radiology. Thanks to imaging excellence, you receive a more precise diagnosis, which then guides a more effective treatment plan.

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