There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to wax and wane. Most women do not visit the doctor because they assume Pagets disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed. Pathophysiology
Pagets disease of the breast is characterised by Paget cells. Paget cells are large cells with clear cytoplasm (clear halo) and eccentric, hyperchromic nuclei found throughout the epidermis.There is some controversy as to whether these cells arise from the ductal system of the breast, or whether these cells are a result of in situ malignant transformation.According to the migratory theory, ductal carcinoma in situ cells migrate into the lactiferous sinuses and the nipple skin. Cancer cells disrupt the normal epithelial barrier and extracellular fluid accumulates on the surface of the skin, resulting in the crusting of the areola skin. Diagnosis
Recommended tests are a mammogram and a biopsy to confirm the diagnosis, and cytopathology may also be helpful. Pagets disease is difficult to diagnose due to its resemblance to dermatitis and eczema; even in patients after ductal carcinoma in situ surgery. Eczema tends to affect the areola first, and then the nipple, whereas Pagets spreads from the nipple.
Invasive cancer or extensive ductal carcinoma in situ is primarily treated with modified radical mastectomies. The procedure consists in the removal of the breast, the lining over the chest muscles and a part of the lymph nodes from under the arm. In cases of noninvasive cancers, simple mastectomies are performed in which only the breast with the lining over the chest muscles is removed. Patients with cancer that has not spread beyond the nipple and the surrounding area are often treated with breast-conserving surgery or lumpectomy. They usually undergo radiation therapy after the actual procedure to prevent recurrence. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer. In most cases, adjuvant treatment is part of the treatment schema. This type of treatment is normally given to patients with cancer to prevent a potential recurrence of the disease. Whether adjuvant therapy is needed depends upon the type of cancer and whether the cancer cells have spread to the lymph nodes. In Pagets disease, the most common type of adjuvant therapy is radiation following breast-conservative surgery. Adjuvant therapy may also consist of anticancer drugs or hormone therapies. Hormonal therapy reduces the production of hormones within the body, or prevents the hormones from stimulating the cancer cells to grow, and it is commonly used in cases of invasive cancer by means of drugs such as tamoxifen and anastrozole.