Causes and risk factors
The exact cause of Paget’s disease is unknown. It is stated that the cancer arises in the breast skin and spreads to the nipple. Some theories suggest that the cancer arises in the breast itself and invades the nipple. It is almost always associated with underlying invasive breast cancer or ductal carcinoma in situ [DCIS]. The disease is common in women and develops after 50 years of age. There are some risk factors which are associated with the disease like overweight women, close relatives with breast cancer, past history of breast cancer, past history of non-cancerous lumps, dense breast tissue, women on hormone replacement therapies, women with early menarche or late menopause, women with first pregnancy after age 30, radiation exposure, alcohol consumption, jobs causing exposure to carcinogens, or endocrine disturbance.
Clinical presentation
Paget’s disease affects the skin of the nipple and shows eczema-like signs and symptoms. Area around nipple is scaly, red, and itchy. There is crusting of skin around the nipple. There is mild irritation of the nipple. The lesion extends to the areola. Patient complains of burning sensation in the nipple. Bleeding from the lesion of the nipple can be seen. Patient experiences tingling, increased sensitivity, and pain in the affected region. There is flattening of nipple. Patients with Paget’s disease of nipple often have a lump in the breast.
Investigation
Medical history by the patient and Clinical examination by the doctor helps in diagnosis. Self-breast examination may reveal the condition. Mammogram is an essential diagnostic tool for early detection of any type of breast cancer. Further investigations include fine needle aspiration cytology [FNAC] which confirms the diagnosis. Examination of nipple discharge is advised. Presence of typical malignant cells known as Paget’s cells in microscopic study is diagnostic of the disease. Imaging studies such as USG of breast, MRI scan of breast is useful for further evaluation.
Treatment
Treatment depends upon the stage of cancer. Modified radical mastectomy is advised in underlying DCIS. Lumpectomy – In this surgery, the surgeon removes the affected part and a small margin of surrounding healthy tissue. Here, the treatment is focused on conservation of breast tissue. It is followed by radiation treatment. In case of underlying invasive carcinoma, mastectomy is performed [a surgery to remove all of the breast tissue]. Most mastectomy procedures remove all of the breast tissue, which include the lobules, ducts, nipple, areola, fatty tissue, and some skin. Supportive care is given to prevent further spread of cancer such as by chemotherapy, hormonal therapy, and targeted cell therapy using medicines.
Other Modes of treatment
The other modes of treatment can also be effective in treating Paget’s disease of the nipple. Homoeopathy is a science which deals with individualization and considers a person in a holistic way. This science can be helpful in combating the symptoms. Similarly, the Ayurvedic system of medicine which uses herbal medicines and synthetic derivates are also found to be effective in treating Paget’s disease of the nipple.
Facts and figures
Paget’s disease of the nipple accounts for about 1% of breast cancers.
