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Paget's Disease of The Nipple (cont.)

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 (1, 2, 3). In a biopsy, the doctor removes a small sample of tissue. A pathologist examines the tissue under a microscope to see if Paget cells are present. The pathologist may use a technique called immunohistochemistry (staining tissues to identify specific cells) to differentiate Paget cells from other cell types (1). A sample of nipple discharge may also be examined under a microscope for the presence of Paget cells (3).

Because most people with Paget's disease of the nipple also have underlying breast cancer, physical examination and mammography (x-ray of the breast) 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 (1, 2, 5). 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 DCIS has been diagnosed (5). In this operation, the surgeon removes the breast, the lining over the chest muscles, and some of 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 (2, 5).

Alternatively, patients whose disease is confined to the nipple and the surrounding area may undergo breast-conserving surgery or lumpectomy followed by radiation therapy (1, 2, 5). 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 (return of the cancer).

During surgery, particularly modified radical mastectomy, the doctor may perform an axillary node dissection to remove the lymph nodes under the arm (1, 5). The lymph nodes are then examined to see if the cancer has spread to them. In some cases, a sentinel lymph node biopsy may be performed to remove only one or a few lymph nodes.

Adjuvant treatment (treatment that is given in addition to surgery to prevent the cancer from coming back) may be part of the treatment plan, depending on the type of cancer and whether cancer cells have spread to the lymph nodes. Radiation treatment is a common adjuvant therapy for Paget's disease of the nipple following breast-conserving surgery. Adjuvant treatment with anticancer drugs or hormone therapies may also be recommended, depending on the extent of the disease and prognostic factors (estimated chance of recovery from the disease or chance that the disease will recur).



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