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 and accounts for about 1% of all breast cancers.
  • Paget's disease of the nipple may be associated with an underlying breast cancer, either intraductal or invasive.
  • Scientists do not know what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops: One theory is that it starts in the skin; the other theory is that it starts in the breast and spreads into the nipple.
  • 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.
  • Mastectomy is the usual treatment for Paget's disease of the nipple. Additional treatments, such as radiation or chemotherapy, may be recommended under certain circumstances.

What Are the Symptoms of Paget’s Disease of the Nipple?

Symptoms of early Paget’s disease of the nipple include redness and mild scaling and flaking of the nipple skin. Early symptoms may cause only mild irritation and may not be enough to prompt a visit to the doctor. Improvement in the skin can occur spontaneously, but this should not be taken as a sign that the disease has disappeared. More advanced disease may show more serious destruction of the skin. At this stage, the symptoms may include tingling, itching, increased sensitivity, burning, and pain. There may also be a bloody discharge from the nipple, and the nipple can appear flattened against the breast.

In approximately half of patients with Paget’s disease of the nipple, a lump or mass in the breast can be felt during a physical exam. In most cases, Paget’s disease of the nipple is initially confined to the nipple, later spreading to the areola (the circular area of darker skin the surrounds the nipple) or other regions of the breast. Paget’s disease of the nipple can also be found only on the areola, where it may resemble eczema, a noncancerous itchy red rash. Although rare, Paget’s disease of the nipple can occur in both breasts.

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How Is Paget’s Disease of the Nipple Diagnosed?

If a health care provider suspects Paget’s disease of the nipple, a biopsy of the nipple skin is performed. In a biopsy, the doctor removes a small sample of tissue that is examined for the presence of Paget's cells.

Because most people with Paget’s disease of the nipple also have underlying breast cancer, a physical exam, mammography (X-ray of the breast), and possible a breast MRI scan are used to make a complete diagnosis.

How Is Paget’s Disease of the Nipple Treated?

Surgery is the most common treatment for Paget’s disease of the nipple. The specific treatment often depends on the characteristics of the underlying breast cancer.

A modified radical mastectomy may be recommended when invasive cancer or extensive ductal carcinoma in situ (DCIS) has been diagnosed. In this operation, the surgeon removes the breast, the lining over the chest muscles, and the lymph nodes under the arm. In cases where underlying breast cancer is not invasive, the surgeon may perform a simple mastectomy to remove only the breast and the lining over the chest muscles.

Alternatively, patients whose disease is confined to the nipple and the surrounding area may undergo breast-conserving surgery or lumpectomy followed by radiation therapy. During breast-conserving surgery, the surgeon removes the nipple, areola, and the entire portion of the breast believed to contain the cancer. In most cases, radiation therapy is also used to help prevent recurrence of the cancer.

WebMD Medical Reference Reviewed by Nivin Todd, MD on July 24, 2016

Sources

SOURCES: 

Kaelin CM. Paget’s Disease. In: Harris JR, Lippman ME, Morrow M, Osborne CK, editors. Diseases of the Breast. 3rd ed. Philadelphia: Lippincott Williams and Wilkins, 2004.

DeVita,VT Jr., Hellman S, Rosenberg SA, editors. Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia: Lippincott Williams and Wilkins, 2004.

Beers MH, Berkow R, editors. The Merck Manual of Diagnosis and Therapy. 17th ed. Whitehouse Station, NJ: Merck & Company, Inc., 1999.

American Cancer Society (2005). Cancer Facts and Figures 2005. Atlanta, GA: American Cancer Society. Retrieved April 20, 2005, from http://www.cancer.org/downloads/STT/CAFF2005f4PWSecured.pdf.

Marcus E. The management of Paget’s disease of the breast. Current Treatment Options in Oncology 2004; 5:153-160.

National Cancer Institute, National Institutes of Health.

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