Paget's Disease of the Nipple: An Overview
Paget's disease of the nipple, also called Paget's disease of the breast, is an uncommon type of cancer that forms in or around the nipple.
More than 95 percent of people with Paget's disease of the nipple also have underlying
breast cancer; however, Paget's disease of the nipple accounts for less than 5 percent of all cases of breast cancer. For instance, of the estimated 211,240 new cases of breast cancer projected to be diagnosed in 2005, fewer than 11,000 were expected to involve Paget's disease of the nipple.
Most patients diagnosed with Paget's disease of the nipple are over age 50, but rare cases have been diagnosed in patients in their 20s. The average age at diagnosis is 62 for women and 69 for men. Paget's disease of the nipple is rare among both women and men.
Paget's disease of the nipple was named after Sir James Paget, a scientist who noted an association between changes in the appearance of the nipple and underlying breast cancer. There are several other unrelated diseases named after Paget, including Paget's disease of the bone and Paget's disease of the vulva.
Causes of Paget's Disease of the Nipple
Scientists do not know exactly what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops.
One theory about the cause of Paget's disease of the nipple proposes that cancer cells, called Paget cells, break off from a tumor inside the breast and move through the milk ducts to the surface of the nipple, resulting in the disease. This theory is supported by the fact that more than 97 percent of patients with Paget's disease also have underlying invasive breast cancer or ductal carcinoma in situ (DCIS). DCIS, also called intraductal carcinoma, is a condition in which abnormal cells are present only in the lining of the milk ducts in the breast, and have not invaded surrounding tissue or spread to the lymph nodes. DCIS sometimes becomes invasive breast cancer. Invasive breast cancer is cancer that has spread outside the duct into the breast tissue, and possibly into the lymph nodes under the arm or into other parts of the body.
The other theory concerning the development of Paget's disease of the nipple suggests that skin cells of the nipple spontaneously become Paget cells. This theory is supported by the rare cases of Paget's disease in which there is no underlying breast cancer, and the cases in which the underlying breast cancer is found to be a separate tumor from the Paget's disease.
Symptoms of Paget's Disease of the Nipple
Early symptoms of Paget's disease of the nipple include:
- Redness around the nipple
- Mild scaling of the nipple skin
- Flaking of the nipple skin.
These early symptoms may cause only mild irritation and may not be enough to prompt a visit to the doctor. Improvement in the skin can occur spontaneously, but this should not be taken as a sign that the disease has disappeared.
More advanced symptoms of Paget's disease of the nipple may show more serious destruction of the skin. At this stage, the symptoms may include:
- Tingling
- Itching
- Increased sensitivity
- Burning
- Pain
- Discharge from the nipple.
The nipple may also appear flattened against the breast.
In approximately half of patients with Paget's disease of the nipple, a lump or mass in the breast can be felt during physical examination. In most cases, Paget's disease of the nipple is initially confined to the nipple, later spreading to the areola or other regions of the breast. The areola is the circular area of darker skin that surrounds the nipple. Paget's disease of the nipple can also be found only on the areola, where it may resemble eczema, a non-cancerous, itchy, red rash. Although rare, Paget's disease of the nipple can occur in both breasts.
Diagnosing Paget's Disease of the Nipple
If a healthcare provider suspects Paget's disease of the nipple, a biopsy of the nipple skin is performed. In a biopsy, the doctor removes a small sample of tissue. A pathologist (someone who studies disease) examines the tissue under a microscope to see if Paget cells are present. The pathologist may use a technique called immunohistochemistry (staining tissues to identify specific cells) to differentiate Paget cells from other cell types. A sample of nipple discharge may also be examined under a microscope for the presence of Paget cells.
Because most people with Paget's disease of the nipple also have underlying
breast cancer, physical examination and mammography (x-ray of the breast) are used to make a complete diagnosis.
Treatment for Paget's Disease of the Nipple
Surgery is the most common treatment for Paget's disease of the nipple. The specific treatment for Paget's disease of the nipple often depends on the characteristics of the underlying
breast cancer.
A modified radical mastectomy may be recommended when invasive cancer or extensive DCIS has been diagnosed. In this operation, the surgeon removes the breast, the lining over the chest muscles, and some of the lymph nodes under the arm. In cases where underlying breast cancer is not invasive, the surgeon may perform a simple mastectomy to remove only the breast and the lining over the chest muscles.
Alternatively, patients whose disease is confined to the nipple and the surrounding area may undergo breast-conserving surgery or lumpectomy followed by radiation therapy. During breast-conserving surgery, the surgeon removes the nipple, areola, and the entire portion of the breast believed to contain the cancer. In most cases of Paget's disease of the nipple, radiation therapy is also used to help prevent recurrence (return of the cancer).
During surgery, particularly modified radical mastectomy, the doctor may perform an axillary node dissection to remove the lymph nodes under the arm. The lymph nodes are then examined to see if the cancer has spread to them. In some cases, a
sentinel lymph node biopsy may be performed to remove only one or a few lymph nodes.
Adjuvant treatment (treatment that is given in addition to surgery to prevent the cancer from coming back) may be part of the treatment plan for Paget's disease of the nipple, depending on the type of cancer and whether cancer cells have spread to the lymph nodes. Radiation treatment is a common adjuvant therapy for Paget's disease of the nipple following breast-conserving surgery. Adjuvant treatment with anticancer drugs or hormone therapies may also be recommended, depending on the extent of Paget's disease of the nipple and prognostic factors (estimated chance of recovery from the disease or chance that the disease will recur).
Paget's Disease of the Nipple: Summary
Important points to remember regarding Paget's disease of the nipple include:
- Paget's disease of the nipple is an uncommon type of cancer that forms in or around the nipple.
- Paget's disease of the nipple is almost always associated with an underlying breast cancer.
- Scientists do not know exactly what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops.
- Symptoms of early-stage Paget's disease of the nipple may include redness or crusting of the nipple skin; advanced symptoms often include tingling, itching, increased sensitivity, burning, or pain in the nipple.
- Paget's disease of the nipple is diagnosed by performing a biopsy.
- Surgery is the usual treatment for Paget's disease of the nipple. Additional treatments may be recommended under certain circumstances.
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