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February 9, 2012

Paget's Disease of The Nipple

Key Points

  • Paget's disease of the nipple is an uncommon type of cancer that forms in or around the nipple.
  • Paget's disease of the nipple is almost always associated with an underlying breast cancer.
  • Scientists do not know what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops.
  • Symptoms of early-stage disease may include redness or crusting of the nipple skin' symptoms of more advanced disease often include tingling, itching, increased sensitivity, burning, or pain in the nipple.
  • Paget's disease of the nipple is diagnosed by performing a biopsy.
  • Surgery is the usual treatment for Paget's disease of the nipple. Additional treatments may be recommended under certain circumstances.
  • Many clinical trials for breast cancer are underway.
  • Patient Discussions: Paget's Disease of The Nipple - Symptoms
  • Find a local Oncologist in your town

What is Paget's disease of the nipple?

Paget's disease of the nipple, also called Paget's disease of the breast, is an uncommon type of cancer that forms in or around the nipple (1, 2, 3). More than 95 percent of people with Paget's disease of the nipple also have underlying breast cancer; however, Paget's disease of the nipple accounts for less than 5 percent of all breast cancers (1). For instance, of the 211,240 new cases of breast cancer projected to be diagnosed in 2005, fewer than 11,000 will also involve Paget's disease of the nipple (4).

Most patients diagnosed with Paget's disease of the nipple are over age 50, but rare cases have been diagnosed in patients in their 20s (1). The average age at diagnosis is 62 for women and 69 for men. The disease is rare among both women and men.

Paget's disease of the nipple was named after Sir James Paget, a scientist who noted an association between changes in the appearance of the nipple and underlying breast cancer (1, 5). There are several other unrelated diseases named after Paget, including Paget's disease of the bone and Paget's disease of the vulva; this fact sheet discusses only Paget's disease of the nipple.

What are the possible causes of Paget's disease of the nipple?

Scientists do not know exactly what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops (1, 2). One theory proposes that cancer cells, called Paget cells, break off from a tumor inside the breast and move through the milk ducts to the surface of the nipple, resulting in Paget's disease of the nipple. This theory is supported by the fact that more than 97 percent of patients with Paget's disease also have underlying invasive breast cancer or ductal carcinoma in situ (DCIS) (1). DCIS, also called intraductal carcinoma, is a condition in which abnormal cells are present only in the lining of the milk ducts in the breast, and have not invaded surrounding tissue or spread to the lymph nodes. DCIS sometimes becomes invasive breast cancer. Invasive breast cancer is cancer that has spread outside the duct into the breast tissue, and possibly into the lymph nodes under the arm or into other parts of the body.

The other theory suggests that skin cells of the nipple spontaneously become Paget cells. This theory is supported by the rare cases of Paget's disease in which there is no underlying breast cancer, and the cases in which the underlying breast cancer is found to be a separate tumor from the Paget's disease (1).



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Paget's Disease of the Nipple

What Is Lymphedema?

Lymphedema is an abnormal buildup of fluid that causes swelling, most often in the arms or legs. The condition develops when lymph vessels or lymph nodes are missing, impaired, damaged, or removed.

There are two types of lymphedema: primary and secondary.

Primary lymphedema is rare and is caused by the absence of, or abnormalities in, certain lymph vessels at birth.

Secondary lymphedema occurs as a result of a blockage or interruption that alters the flow of lymph through the lymphatic system and can develop from an infection, cancer, surgery, scar tissue formation, trauma, deep vein thrombosis (a blood clot in a vein), radiation, or other cancer treatment.

Who Is at Risk for Developing Lymphedema?

People who have had any of the following procedures may be at risk for developing lymphedema:

  • Simple mastectomy in combination with axillary (arm pit) lymph node removal./l...

Read the Breast Cancer and Lymphedema article »







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