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Paget’s disease is a rare form of breast cancer which causes skin changes in the nipple area (bleeding, itching, flaking, and nipple discharge) and accounts for fewer than 3% of all breast cancers. Often, Paget’s is mistaken for eczema or an infection before the correct diagnosis is made.
Under the microscope, Paget’s cells are often found to be high grade, which means that they look very different from normal cells, and are dividing rapidly. About half are found to be positive for estrogen and progesterone receptors, and most are positive for the HER2 protein.
An important part of the diagnosis and staging of Paget’s disease includes checking for another tumor elsewhere in the breast. Most of the time, there is another cancer, even if it is not seen right away on a mammogram, so other tests may be done to be sure. Staging for Paget’s is done based on the size of the breast tumor, not the skin changes in the nipple area. If, after a full evaluation, cancer is found only in the nipple area, it is staged as a Stage 0 cancer.
Treatment of Paget's Disease is similar to that of more common types of breast cancer, including local therapy and, sometimes, systemic therapy.
Local therapy is aimed at preventing the cancer from coming back in the breast. Local therapy includes surgery (lumpectomy or mastectomy) and may include radiation.
Systemic therapy is used to prevent the disease from coming back or spreading to another part of the body. This may include endocrine (hormone) therapy, chemotherapy, and therapy that targets the HER2 protein. Often, different types of treatment are used together to achieve the best result.
Your treatment plan will be based on the features of the tumor (type, tumor grade, hormone receptor status, and HER2 status and the stage of the disease (tumor size and node status). Your oncology team will work together to recommend a treatment plan based on what is known about Paget’s disease in general and tailored specifically to your disease.