What is Paget's nipple disease?

Paget's disease of the nipple should not be confused with other forms of Paget's disease, particularly Paget's disease of the bone or vulva. All three conditions share a common identification and description by the 19th century British physician Sir James Paget. Each of them is a separate disease, named for the pioneering work of this doctor. Paget's nipple disease is actually a rare form of breast cancer, accounting for only about 5% of all breast cancer cases, and is most often seen in women over the age of 60. Occasionally younger women get the condition, but it is very rare.

In most cases of Paget's nipple disease, the most noticeable factor is that the nipple is covered with a red, scaly rash. Most women ignore this early rash, but if it continues for more than a few days, women should see a doctor. The rash may begin to crust over and may spread from the nipple into the surrounding skin. While the early rash is not particularly bothersome, as the disease progresses the rash may itch, sting or burn, creating considerable discomfort.

Upon examination, half of all people diagnosed with Paget's disease of the nipple have a palpable lump below the nipple. In all, 95% of people with the condition have a tumor in their breast tissue, usually located near the milk ducts. When a tumor cannot be felt, patients undergo mammography and sometimes ultrasound of the breast tissue to locate and identify tumors. What creates some medical mystery is that in about 5% of cases, no tumor is present. However, even without an underlying cancerous growth, the tissue in the nipple is cancerous and can possibly spread. All cases are treated to prevent spread.

There are several treatment options. For some women, the malignant growths are not only under the nipple but have spread to under the arms. A complete mastectomy may be required. Others have a lumpectomy and remove any nipple or surrounding tissue that has Paget cells. Radiation therapy after lumpectomy or mastectomy is standard, and women may also begin hormone suppression therapy through oral medications or chemotherapy to prevent recurrence.

The survival rate for Paget's nipple disease depends on the stage at which the cancer is identified. If the condition affects only the nipple tissue, the survival rate is 99.5%. When there are underlying tumors, the survival rate is assessed by the stage and size of the tumor and how far it may have invaded. In Stage One, a breast tumor is no larger than about 0.8 inches (2,032 cm) and cancer cells are not present in the lymph nodes. About 80% of people in this stage are completely cured.

Stage two means that the cancer has spread to the lymph nodes and the size of the tumor is no larger than about two inches (5.08 cm). The survival rate can be determined by the extent to which the cancer affects the lymph nodes, but the overall survival rate for people diagnosed at Stage Two is 70%. Patients in stage three have a 40% survival rate five years after diagnosis with treatment, and patients in stage four have a much lower rate, a 20% survival rate at five years.

There are investigational treatments and clinical trials for Paget's nipple disease, and the prospects for improving survival rates are good. As with any breast cancer, early detection is key to achieving higher survival rates. Pay attention to any rash that forms on the nipple and do monthly self-exams to check for lumps.

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