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Paget Disease of the Breast

Paget disease of the breast facts*

*Paget disease of the breast facts medically reviewed by Melissa Conrad Stöppler, MD, on 1/21/2015

  • Paget disease of the breast is a form of breast cancer that affects the skin of the nipple.
  • The cause of Paget disease of the breast is poorly understood, but it may be related to tumor cells from other sites in the breast traveling through the milk ducts to the nipple.
  • Symptoms and signs of Paget disease of the breast can be similar to those of benign skin conditions and can include itching, redness, thickening, or crusting of the skin.
  • Diagnosis of Paget disease of the breast is confirmed by a biopsy, a sample of tissue taken for laboratory analysis and diagnosis. The hallmark sign of Paget disease of the breast is the presence of certain malignant cells known as Paget cells, which are visible on microscopic examination of the tissue biopsy.
  • Treatment of Paget disease of the breast typically involves surgery (either breast-conserving surgery or mastectomy). Radiation therapy is given following breast-conserving surgery.
  • Depending upon the specific characteristics of the tumor, adjuvant therapy consisting of chemotherapy and/or hormonal therapy, may be given.
  • The prognosis and survival rate for Paget disease of the breast depend upon the extent of spread of the cancer and the presence or absence of malignant tumors in the same breast.
  • The five-year survival for all women in the U.S. who were diagnosed with Paget disease of the breast between 1988 and 2001 was 82.6%.

Paget disease of the breast key points

  • Paget disease of the breast is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, known as the areola.
  • Most of the time, people with Paget disease of the breast also have one or more tumors inside the same breast.
  • Paget disease of the breast may be misdiagnosed at first because its early symptoms are similar to those caused by some benign skin conditions.
  • The outlook for people diagnosed with Paget disease of the breast depends on a variety of factors, including the presence or absence of invasive cancer in the affected breast and, if invasive cancer is present, whether or not it has spread to nearby lymph nodes.

What is Paget disease of the breast?

Paget disease of the breast (also known as Paget disease of the nipple and mammary Paget disease) is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, which is called the areola. Most people with Paget disease of the breast also have one or more tumors inside the same breast. These breast tumors are either ductal carcinoma in situ or invasive breast cancer.

Paget disease of the breast is named after the 19th century British doctor Sir James Paget, who, in 1874, noted a relationship between changes in the nipple and breast cancer. (Several other diseases are named after Sir James Paget, including Paget disease of bone and extramammary Paget disease, which includes Paget disease of the vulva and Paget disease of the penis. These other diseases are not related to Paget disease of the breast. This fact sheet discusses only Paget disease of the breast.)

Malignant cells known as Paget cells are a telltale sign of Paget disease of the breast. These cells are found in the epidermis (surface layer) of the skin of the nipple and the areola. Paget cells often have a large, round appearance under a microscope; they may be found as single cells or as small groups of cells within the epidermis.


Source: MedicineNet.com
http://www.medicinenet.com/pagets_disease_of_the_nipple/article.htm

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