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Paget disease of the nipple

Paget disease of the nipple (or Paget disease of the breast) is a rare type of breast cancer that appears as a rash or other skin changes on the nipple. It rarely occurs in both breasts. It is more common in women over the age of 50.

It is thought that this type of cancer starts in the ducts of the breast and the cancer cells move through the ducts to the skin of the nipple. The cancer may spread to the areola (the dark-coloured skin around the nipple). Most women with Paget disease also have an underlying breast cancer, either ductal carcinoma in situ (DCIS) or invasive ductal carcinoma.

Signs and symptoms

The signs and symptoms of Paget disease of the nipple may include changes to the breast, such as:

  • nipple skin changes
    • crusting
    • scaling or flaking
    • redness of the nipple and areola
  • burning
  • itching
  • pain
  • nipple discharge
  • bleeding from the nipple
  • flattened nipple
  • inverted nipple (turned inward)
  • a lump in the breast
    • About half of people with Paget disease have a lump in the area near the nipple.

Diagnosis

If the signs and symptoms of Paget disease of the nipple are present, or if the doctor suspects Paget disease of the nipple, tests will be done to make a diagnosis. Tests may include:

Treatment

Treatment for Paget disease of the nipple often depends on whether or not there is an underlying breast cancer, and whether or not the cancer has spread to the lymph nodes. Treatment is similar to the more common types of breast cancer.

Treatment options for Paget disease of the nipple may include:

  • surgery
    • A mastectomy is done if there is an underlying invasive breast cancer.
    • If the disease is confined to the nipple, the doctor may consider breast-conserving surgery to remove the nipple, areola and surrounding tissue.
    • An axillary lymph node dissection may be done if there is concern that the cancer has spread to the lymph nodes.
  • radiation therapy
    • External beam radiation therapy is given after breast-conserving surgery to reduce the chance of the cancer recurring in the breast.
    • It is also given if the cancer has spread to the lymph nodes.
  • hormonal therapy
  • chemotherapy
    • Chemotherapy may be offered for women with Paget disease of the nipple who have an underlying invasive breast cancer that has spread to the lymph nodes.
    • Drugs and combinations are the same as used for breast cancer.

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