Paget disease of the breast occurs when cancer cells within the breast grow along the sides of the milk ducts and exit onto the skin of the nipple and surrounding circle of skin called the areola. It can arise at any age associated with breast cancer and usually involves a single breast.
Although the hallmarks of the disease are abnormalities in the surface of the nipple and areola, Paget disease of the breast is not a form of skin cancer but the product of a malignancy originating deeper in the breast.
What are the symptoms of Paget disease?
The most common symptoms of Paget disease of the breast are:
- Irritation of the skin of the nipple and areola;
- Inflammation and oozing – known as an ulceration – of the nipple and areola, especially if the ulceration doesn’t heal;
- Itching, tingling, or redness in the area.
Because these symptoms can also be signs of readily treated skin conditions such as eczema, it’s important to have them examined if they persist for more than a couple weeks.
How is Paget disease of the breast diagnosed?
The first step in diagnosing the disease is a mammogram and ultrasound exam of both breasts to find if an underlying tumor is present. If it is, doctors will perform a biopsy in which cells from the nipple and the underlying breast tissue abnormality are removed for examination by a pathologist. Based on the appearance of the cells under the microscope, the pathologist will determine if they’re cancerous.
How is it treated?
Fortunately, most cases of Paget disease of the breast are detected when the cancer is in an early stage of development. Many cases involve ductal carcinoma in situ (DCIS), also known as stage 0 cancer, in which abnormal cells are confined to the lining of a milk duct. Though not in itself cancerous, DCIS increases the risk that an individual will develop invasive breast cancer.
Treatment for Paget disease of the breast is based on the extent of the cancer as well as its specific type. Small tumors can often be treated by a central lumpectomy, breast-conserving surgery that removes only the tumor and a small amount of surrounding tissue as well as the nipple and areola, followed by radiation therapy to the breast. Larger tumors may require a mastectomy, or removal of the entire breast. Because of the presence of cancer cells in the nipple, both lumpectomy and mastectomy require removal of the nipple.
Patients whose cancer has spread to lymph nodes in the breast may have those and other lymph nodes removed. Further treatment is geared to the stage of the cancer — whether it has metastasized to other parts of the body — and other factors such as whether the tumor cells are driven to grow by estrogen and progesterone, and whether they overproduce the HER2 protein. Chemotherapy and/or therapies targeting specific molecular abnormalities in the tumor cells may be recommended.
When the cancer underlying Paget disease of the breast is diagnosed at an early stage, as it commonly is, treatment is usually very effective, with about 90% of patients being cured. When the disease has had a chance to progress beyond this stage, success rates of treatment decline, underscoring the importance of having changes in the nipple examined by a physician at an early stage.
About the Medical Reviewer
Dr. Nakhlis received her medical degree from Rush Medical College in Chicago in 1996. She subsequently completed her residency in general surgery and her breast surgery fellowship at Northwestern University in Chicago. She is an attending physician in the Brigham and Women's Hospital and Brigham and Women's Faulkner Hospital in Boston. Her clinical research interests are focused on lobular neoplasia and other premalignant proliferative breast lesions, and inflammatory breast cancer.