Paget's Disease of Bone: A Rare But Treatable Type of Breast Cancer

Paget's disease of the breast is a rare form of breast cancer named after the 19th-century British doctor Sir James Paget. Although Paget's disease can affect other parts of the human body, Paget's disease of the breast is typically clinically observed with scaling of the nipple skin, nipple redness, or nipple burning. Paget's disease of the breast accounts for 1-4 percent of breast cancer cases. The median age of diagnosis is 57, but cases have been found in patients as young as their early 20s and as old as their late 80s.

It is essential to note that most cases of nipple skin scaling and/or burning are due to benign conditions. If an individual experiences these symptoms, it is crucial to contact a breast-dedicated physician to ensure proper diagnosis is provided.

Diagnosing Paget's Disease

The diagnosis of Paget's disease is relatively straightforward. At Breastlink, our breast-dedicated physicians utilize a simple skin punch biopsy technique to remove a small piece of tissue from the area of the nipple affected by abnormal changes. This procedure is painless, performed under local anesthesia, and takes only a few minutes. A small amount of skin and underlying tissue is removed and sent to a pathologist. In our experience, with hundreds of cases, this method has proven to be highly accurate.

The first step in evaluating a patient with a change in the skin of the nipple is to obtain a detailed history and conduct a careful breast examination, followed by a diagnostic mammogram. If Paget's disease is diagnosed, the next step is to identify the location of the underlying breast cancer.

More than 50 percent of patients with Paget's disease will have an abnormality on their mammogram. When performing a diagnostic mammogram, the mammogram technologist is informed about the possibility of a Paget's disease diagnosis. Special films are used to look for fine calcifications behind the nipple.

If the mammogram is negative and our clinical suspicion is low, we usually suggest that the patient use a cortisone cream on the nipple for two weeks. If the symptoms persist or there is a clinical concern, additional imaging techniques such as breast ultrasound and/or MRI may be ordered. Careful follow-up is always necessary with a potential diagnosis of Paget's disease.

Our Approach to Benign Nipple Conditions

There are various conditions that can mimic Paget's disease, such as atopic dermatitis, nipple eczema, psoriasis, nipple adenoma, or contact dermatitis. Our medical team will address your questions about these topics during an office visit.

In the collective experience of our breast-dedicated medical team, most patient referrals with a suspicion of Paget's disease diagnosis exhibit similar changes in both nipples. However, Paget's disease typically affects only one side. Patients demonstrating bilateral nipple changes are usually referred to a dermatologist who will evaluate the underlying cause of the nipple change if the mammogram is normal.

The majority of patients who undergo a skin punch biopsy receive a benign result. These patients need reassurance that no cancer is present at the time of the office visit. Follow-up observation with the referring doctor is recommended.

At times, a moisturizing cream can help control nipple dryness. Patients with persistent symptoms after a negative biopsy are also referred to a dermatologist for further treatment.

Treatment of Paget’s Disease

The traditional, standard treatment for Paget's disease of the breast was a mastectomy. However, advancements in surgical techniques and equipment have led modern medical practice to favor breast-conserving procedures. A Breastlink surgeon will remove the affected nipple and surrounding breast tissue containing cancer. Once clear surgical margins are secured, the affected breast tissue is irradiated.

Our outcomes with this approach have been outstanding. In rare cases, more extensive involvement of the breast tissue may require a mastectomy, which is typically followed by immediate breast reconstruction (Reconstructive Surgery).