Paget's disease of the breast or nipple is a rare type of breast cancer that occurs in women and men. It develops in and around the nipple, and usually signals the presence of breast cancer beneath the skin.
The disease is named after Sir James Paget who first reported the link between changes in the nipple and underlying breast cancer.
Most cases of Paget's disease are found in menopausal women, but it can also (rarely) appear in women as young as 20.
In the following article, we will look at the causes, diagnosis, and treatment of Paget's disease.
Contents of this article:
Fast facts on Paget's disease of the breast
Here are some key points about Paget's disease of the breast. More detail and supporting information is in the main article.
- Paget's disease of the breast is a rare condition
- The majority of cases of Paget's disease of the breast are a sign of underlying breast cancer
What is Paget's disease of the breast?
Paget's disease indicates a 90 percent chance of an underlying breast cancer.
Paget's disease indicates underlying breast cancer; it was initially believed that the affected cells were not cancerous, but it was later proven that Paget's cells were indeed malignant.
Since the condition is often limited to a surface appearance and appears innocuous, it is sometimes dismissed; however, the condition indicates the presence of a breast cancer that may prove fatal if left untreated.
Most patients diagnosed with Paget's disease of the breast are over the age of 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. However, the disease is rare in both sexes.
Symptoms of Paget's disease of the breast
On average, a woman may experience signs and symptoms for 6-8 months before a diagnosis is made.
Symptoms of Paget's disease vary based on the stage of the disease. The main symptoms are flaky or scaly skin on the nipple, straw-colored or bloody nipple discharge, skin and nipple changes in only one breast, or flattened nipples.
The earliest symptom of Paget's disease is often an eczema-like rash, usually affecting only one nipple. The skin of the nipple and areola may become red, itchy, and inflamed. Some women have an itching or burning sensation.
Fluid discharge might leak from the abnormal area of cells. The nipple might invert inwards. There may or may not be a lump in the breast, and there can be redness, oozing, and crusting. One may also notice a sore that does not heal.
The symptoms usually affect the nipple initially, then spread to the areola and finally the breast. It is common for the symptoms to disappear temporarily, which may falsely lead the patient to believe that the disease has resolved.
Most women do not visit the doctor because they mistake the condition for contact dermatitis or eczema. Women who feel a lump or notice skin irritation that does not heal for over a month are advised to seek the opinion of a specialist.
Patients may also experience crusty, oozing, or hardened skin resembling eczema on the nipple, areola, or both. The skin changes may fluctuate early on, making it appear as if the skin is healing on its own. Some patients complain of burning sensations in the nipples or breasts. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to visit a doctor.
Lumps or masses in the breast occur in 50 percent of patients. In more advanced stages, the disease may cause tingling, increased sensitivity, and pain.
Causes of Paget's disease of the breast
If invasive breast cancer or ductal carcinoma in situ (DCIS) is already present in the breast as a tumor, cells might drift off from the tumor and float up through the milk ducts, where they enter the nipple and areola.
In a few cases of Paget's disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. Researchers suggest that in those cases, nipple skin cells may spontaneously change into cancer cells.
Diagnosing Paget's disease of the breast
One of the most common methods of diagnosing Paget's disease of the breast is a mammogram.
Recommended tests are a mammogram and a biopsy to confirm the diagnosis.
Paget's disease is difficult to diagnose due to its resemblance to dermatitis and eczema. The latter, unlike Paget's disease, usually affects the areola first and then the nipple.
During a physical examination, the doctor examines the unusual areas of the breast, especially the appearance of the skin on and around the nipples and feels for any lumps or areas of thickening.
The most commonly used test to diagnose Paget's disease is the biopsy. A biopsy involves removing a tissue sample from the affected area and examining it under a microscope.
A pathologist may use a technique called immunohistochemistry (staining tissues to identify specific cells) to differentiate Paget cells from other cell types.
Samples of nipple discharge may also be examined under the microscope to check if Paget cells are present.
Imprint or scrape cytology can also be useful; this consists of scraping cells from the affected area, or pressing them onto a glass slide to be examined under the microscope.
Treatment options for Paget's disease of the breast
Surgery is the most common treatment for Paget disease of the breast. The specific treatment often depends on the characteristics of the underlying breast cancer.
A modified radical mastectomy may be recommended when invasive cancer or ductal carcinoma in situ (DCIS) has been diagnosed. In this operation, a 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, a surgeon removes the nipple, areola, and the entire portion of the breast believed to contain the cancerous cells. In most cases, radiation therapy is also used to help prevent the cancer from returning.
Preventing Paget's disease of the breast
Paget's disease of the breast cannot be prevented. However, one may prevent complications of Paget's disease of the breast, such as osteoarthritis, by taking certain medications, maintaining a healthy weight, and regularly exercising.