Main Category: Breast Cancer
Also Included In: Cancer / Oncology
Article Date: 21 Jun 2010 – 0:00 PST
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Paget’s disease of the nipple or breast is a rare type of breast cancer, which can occur in women and men. It shows up in and around the nipple, and usually signals the presence of breast cancer beneath the skin.
Most cases are found in menopausal women, but can also appear in women that are as young as 20.
The disease is named for Sir James Paget. He reported on the link between changes in the nipple and the underlying breast cancer. Although Paget believed the affected cells were not cancerous, it was later proved that the cells were themselves malignant, in addition to indicating underlying breast cancer. Since the condition is often innocuous and limited to a surface appearance, it is sometimes dismissed, despite the fact that it is indicative of breast cancer that may prove fatal if left untreated.
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 breast cancers.
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. The disease is rare among both women and men.
In an interesting note, retired Boston Red Sox center fielder Dom DiMaggio suffered from Paget’s disease and served as a member of the board of directors of the Paget Foundation.
What are the symptoms of Paget’s disease?
A symptom is something the patient senses and describes, while a sign is something other people, such as the doctor notice. For example, drowsiness may be a symptom while dilated pupils may be a sign.
On average, a woman may experience signs and symptoms for six to eight months before a diagnosis is made.
Paget’s disease’s symptoms may vary based on the stage of the disease. However, the main symptoms that can occur in Paget’s disease include flaky or scaly skin on the nipple, straw-colored or bloody nipple discharge, skin and nipple changes in only one breast or the flattened nipples.
The first symptom is usually an eczema-like rash, usually only affecting one nipple. The skin of the nipple and areola may be red, itchy and inflamed. Some women have an itching or burning sensation. Fluid discharge may leak from the abnormal area of cells. The nipple may turn inwards. There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.
The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common that the symptoms disappear for a while and this may be tricky as the patient takes it as a sign that the disease has cured, which is not true.
Most women do not visit the doctor because they mistake it as contact dermatitis or eczema. Women who feel a lump or notice skin irritation that does not seem to heal for over a month are recommended to seek the opinion of a specialist.
Patients may also experience crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both and fluctuating skin changes early on, making it appear as if the skin is healing on its own. Some patients complain of burning sensations on the nipples or breasts. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to consult.
Lumps or masses in the breast occur in 50% of the patients. In more advanced stages, the disease may cause tingling, increased sensitivity and pain.
What are the causes of Paget’s disease?
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. h2 class=”blue_sea_paddingtop”>Diagnosing Paget’s disease
Recommended tests are a mammogram and a biopsy to confirm the diagnosis, and cytopathology may also be helpful.
Paget’s disease is difficult to diagnose due to its resemblance to dermatitis and eczema. The difference between these two types of conditions consists of the detail that the latter, unlike Paget’s disease, rather affect 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 feeling for any lumps or areas of thickening.
The most commonly tests used to diagnose Paget’s disease is the biopsy. A biopsy consists in the removal of a tissue sample from the affected area which is after looked at under the microscope by a pathologist. The 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 may be useful. They consist in scraping cells from the affected area, or pressing them onto a glass slide to be examined under the microscope.
What are the treatment options for Paget’s disease?
Surgery is the most common treatment for Paget disease of the nipple. The specific treatment 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, 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 cancer. In most cases, radiation therapy is also used to help prevent recurrence.
Preventing Paget’s disease
Paget’s disease cannot be prevented. However, one may prevent complications of Paget’s disease, such as osteoarthritis, by taking medicine, staying at a healthy weight, and regularly doing gentle exercise that does not cause stress to the bone.