This page was reviewed under our medical and editorial policy by
Daniel Liu, MD, Plastic and Reconstructive Surgeon
This page was reviewed on February 7, 2022.
Paget’s disease of the breast affects about 1 to 4 percent of breast cancer patients, according to the National Cancer Institute (NCI). It's a rare type of breast cancer that forms in the nipple and areola (the darker, pigmented skin surrounding the nipple).
In most cases, people with Paget’s disease also have ductal carcinoma in situ (DCIS), which is a breast cancer contained within the milk ducts, or invasive breast cancer. However, Paget’s disease is different from other breast cancers in that the cancerous cells are found in the skin of the breast, instead of within the breast.
This condition occurs most often in women, and the average age at diagnosis is 57, though it may affect anyone from teens up to people in their late 80s.
In most cases, Paget’s disease affects one breast, and the symptoms may come and go. This means symptoms may cycle through, appearing to improve and then worsen again. The symptoms may look similar to eczema or other such skin conditions.
Patients may notice:
If Paget’s disease is suspected, doctors may recommend a breast biopsy, in which a small sample of tissue is removed for testing. Under a microscope, these cancer cells appear large and round, and may either be grouped together or alone as single cells. In some circumstances, doctors may have to remove the entire nipple for the biopsy.
Because Paget’s disease may also coexist with other cancer cells within the breast, additional breast cancer screenings may be necessary, such as a mammogram, ultrasound or MRI (magnetic resonance imaging) of the breast—or a combination of these.
As many as half of Paget’s disease patients also have a palpable breast lump, according to the NCI, so a physical breast exam may be a part of the diagnosis process.
Surgery is the primary treatment option for Paget’s disease. Doctors may recommend a mastectomy or lumpectomy, depending on how much of the breast is affected by cancer.
If there’s limited involvement of the breast tissue, a breast-conserving surgery (lumpectomy) may be performed to leave as much of the breast untouched as possible. With this type of surgery, the nipple and areola are removed, along with malignant tissue, typically a small area of noninvasive breast cancer such as ductal carcinoma in situ (DCIS). However, if underlying invasive cancer is found in the breast, in addition to Paget’s disease, a total mastectomy may be more likely.
A minority of patients with Paget's disease have cancer confined to the nipple itself, with no sign of cancer elsewhere in the breast. For these patients, surgical removal of the nipple and areola is typically recommended.
During surgery for a Paget’s disease patient also has invasive breast cancer, doctors routinely check the lymph nodes to ensure there are no cancer cells there that require removal.
Both a mastectomy and lumpectomy are typically followed by radiation therapy for breast cancer, but breast cancer chemotherapy may be recommended as well.
Survival rates differ depending on the severity of Paget’s disease. The five-year relative survival rate for women with Paget’s disease in the United States between 1988 and 2001 was 82.6 percent, according to the NCI. As current treatments change, the overall outlook may improve.
Since Paget’s disease is rare, randomized controlled clinical trials are hard to perform. But patients researching this option have a database available of NCI-supported clinical trials.