Paget's Disease of the Breast
Paget's disease of the breast, also known as Paget disease of the nipple or mammary Paget disease, is a rare form of breast cancer that represents less than 5 percent of breast cancer cases.
The condition starts in or around the nipple in the areola (dark area of skin surrounding the nipple). Cancer cells within the skin of the nipple cause redness and mild scaling and flaking of the nipple skin and may resemble eczema, a non-cancerous skin condition.
Most women with Paget's disease of the nipple also have either ductal carcinoma in situ (DCIS) or invasive breast cancer elsewhere in the breast. Diagnosis is made with a biopsy of the skin of the nipple.
Symptoms of Paget's disease of the breast can be very similar to benign skin conditions. If a patient notices any of the following symptoms, they should make an appointment with their physician immediately:
- Itching or tingling of the nipple or areola
- Flaking, crusty, or thickened skin on or around the nipple
- A flattened nipple
- Discharge from the nipple that may be yellowish or bloody
- A lump in the breast
Causes and Risk Factors
The cause of Paget's disease of the breast is unknown, although there does appear to be a link between an underlying ductal cancer and the disease.
Risk factors include:
- Age — risk of developing the condition increases with age
- A history of benign breast disease
- A personal history of breast cancer
- A family history of breast cancer
- Dense breast tissue
- Exposure to radiation
- Race — Caucasian women have a higher risk of breast cancer
- Using combination estrogen-progestin hormone replacement therapy after menopause
When diagnosing Paget’s disease of the breast, the physician will often start with a clinical breast exam to feel for any lumps or unusual skin changes in the breast or nipple. A mammogram is also used to see the tissue inside the breast.
If the medical team sees evidence of abnormal cell growth during the mammogram, a biopsy will be performed. During a biopsy, a sample of the abnormal tissue will be taken using a needle. The doctor may use stereotactic images, such as mammography or ultrasound, to guide the needle to the area of concern. A specialist, known as a pathologist, will examine the tissue sample under a microscope and determine if cancer is present.
Treatment of Paget's disease of the breast will generally begin with surgery. Once the surgery has been performed, additional treatments may also be used, including:
- Surgery. Surgery to remove the affected tissue may include breast-sparing techniques or the removal of the entire breast. The type of surgery performed will depend on the stage and spread of the cancer. A lumpectomy, which removes the affected tissue while preserving as much of the natural breast tissue as possible, is the most conservative option and is generally paired with radiation therapy.
For some patients, a mastectomy, a procedure that removes all of the breast tissue, may be the best course of treatment. There are different forms of mastectomy surgeries including nipple-sparing and skin-sparing options.
Removing the lymph nodes in the underarms may also be needed. This can be done as either a sentinel lymph node biopsy or axillary lymph node dissection.
- Radiation therapy. Additional treatments may include radiation therapy, which uses high-energy radiation to kill the cancer cells. The radiation may be directed from outside the body (external) or it may come from an implant placed inside the breast.
- Chemotherapy. Chemotherapy uses drugs to kill the cancer cells. The drugs may be provided through a pill or through an IV.
- Hormone therapy. Estrogen has been associated with breast cancer and for some patients hormone therapy may be an option. This method of treatment blocks estrogen from reaching the breast tissue.