What Is Paget's Disease Of The Breast?
Paget's disease of the breast, also known as Paget's disease of the nipple, is a rare form of breast cancer, with symptoms such as burning sensations.
Paget’s disease of the breast is a rare form of breast cancer in which cancer cells collect in or around the nipple. The cancer usually affects the ducts of the nipple first (small milk-carrying tubes). Then it spreads to the nipple surface and the areola (the dark circle of skin around the nipple). The nipple and areola often become scaly, red, itchy, and irritated. The disease is named after British doctor Sir James Paget. In 1874, he noted a relationship between changes in the nipple and breast cancer.
According to the National Cancer Institute, Paget’s disease of the nipple accounts for less than 5% of all breast cancer cases in the world. More than 97% of people with Paget’s disease also have cancer, somewhere else in the breast. The unusual changes in the nipple and areola are often the first indication that breast cancer is present.
Who gets Paget’s disease of the breast?
Paget disease of the breast occurs in both women and men, but most cases occur in women. Approximately 1 to 4 percent of all cases of breast cancer also involve Paget disease of the breast. The average age at diagnosis is 57 years, but the disease has been found in adolescents and in people in their late 80s.
What causes Paget’s disease of the breast?
Doctors don’t fully understand what causes the disease. The general theory is that cancer cells from a tumor inside the breast travel through the milk ducts to the nipple and areola. This explains why Paget’s disease and tumors inside the same breast are almost always found together.
Another theory is that cells in the nipple or areola become cancerous on their own. This would explain why a few people develop Paget’s disease of the breast without having a tumor inside the same breast.
What are the symptoms the disease?
The symptoms are often mistaken for those of some benign skin conditions, such as dermatitis or eczema. These symptoms include:
- Itching, tingling, or redness in the nipple and/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
Because the early symptoms of Paget disease of the breast may suggest a benign skin condition, and because the disease is rare, it may be misdiagnosed at first. People with Paget disease of the breast have often had symptoms for several months before being correctly diagnosed.
A nipple biopsy allows doctors to correctly diagnose Paget disease of the breast. There are several types of nipple biopsies, including the procedures described below.
- Surface biopsy: A glass slide or other tool is used to gently scrape cells from the surface of the skin.
- Shave biopsy: A razor-like tool is used to remove the top layer of skin.
- Punch biopsy: A circular cutting tool, called a punch, is used to remove a disk-shaped piece of tissue.
- Wedge biopsy: A scalpel is used to remove a small wedge of tissue.
In some cases, doctors may remove the entire nipple. A pathologist then examines the cells or tissue under a microscope to look for Paget cells.
Most people who have Paget disease of the breast also have one or more tumors inside the same breast. In addition to ordering a nipple biopsy, the doctor should perform a clinical breast exam to check for lumps or other breast changes. As many as 50 percent of people who have Paget disease of the breast have a breast lump that can be felt in a clinical breast exam. The doctor may order additional diagnostic tests, such as a diagnostic mammogram, an ultrasound exam, or a magnetic resonance imaging scan to look for possible tumors.
For many years, mastectomy was the standard surgery for Paget disease of the breast. Doctors did this type of surgery because patients almost always had one or more tumors in the same breast.
Studies have shown, however, that breast-conserving surgery is a safe option. That includes removal of the nipple and areola, followed by whole-breast radiation therapy. But these patients must not have a palpable lump in their breast. And their mammograms must not reveal a tumor.
Patients who have a breast tumor and are having a mastectomy should have a sentinel lymph node biopsy. This is to see whether the cancer has spread to the axillary lymph nodes. If cancer cells are found in the sentinel lymph node(s), more extensive axillary lymph node surgery may be needed. Depending on the stage and other features of the underlying breast tumor, adjuvant therapy, consisting of chemotherapy and/or hormonal therapy, may also be recommended.