Paget’s disease of the breast is a rare cancer of the nipple and areola that is often associated with an invasive type of cancer called ductal carcinoma in situ.
Paget’s disease occurs in about 1% to 4% of all breast cancers. It most commonly affects women between the ages of 40 and 60, especially during menopause. The disorder can also appear in adolescents and older adults, and in rare cases, in men.
Paget’s disease is named after Sir James Paget, an English surgeon in the 1800s who published articles on the link between nipple changes and underlying breast cancer.
Several unrelated disorders are also named after Paget, including a bone disorder called Paget’s disease of bone and extramammary Paget’s disease, which includes Paget’s disease of the vulva and Paget’s disease of the penis. This article covers Paget disease of the breast only.
This article will discuss symptoms, causes, treatment options, and when to see a healthcare provider.
Paget’s disease causes red, painful, flaky, and scaly skin on and around the nipple. In the early days, these symptoms may come and go.
Symptoms usually get worse over time and may include:
- Itching or burning sensation in the nipple and/or areola
- peeling, crusting, or thickening of the skin on or around the nipple
- pain and sensitivity
- nipple flattening
- yellow or bloody discharge from the nipple
- lump in the affected area
Symptoms of Paget’s disease are sometimes mistaken for skin conditions, including eczema or dermatitis. Paget’s disease is usually found in only one breast and can be improved with topical medications – potentially delaying an accurate diagnosis.
When to see a healthcare provider
If you develop any of the signs and symptoms of Paget’s disease, seek medical attention as soon as possible. Early diagnosis increases the chances of successful treatment.
The diagnosis of Paget’s disease may involve the following steps:
- Clinical Breast Exam: Up to 50% of people with Paget’s disease of the nipple will feel a breast lump or thickened area during a clinical exam.
- Mammogram: If you have Paget’s disease of the breast, a mammogram may show nipple and skin changes associated with underlying breast cancer. Your healthcare provider may also follow up with magnetic resonance imaging (MRI).
- Breast biopsy: During a breast biopsy, a small tissue sample is taken from the lump, skin, or nipple for microscopic examination. If there is nipple discharge, it will also be collected and checked.
- Sentinel lymph node biopsy: In this procedure, the surgeon locates and removes the sentinel lymph node—where breast cancer cells go first. If sentinel lymph nodes are checked and found to be negative, cancer is unlikely to be found in the remaining lymph nodes.
If cancer cells are detected in any biopsy sample, you will need treatment.
The cause of Paget’s disease of the breast is unknown. According to the American Cancer Society, about 80 to 90 percent of Paget patients also have DCIS or invasive cancer elsewhere in the breast.
Cancer cells may travel up the duct from the tumor and into the nipple and areola.
Sometimes there is no underlying breast cancer, or if a tumor is present, it is not related to nipple disease. The researchers believe that under these conditions, papillary skin cells may spontaneously turn into cancer cells.
The same factors that increase the risk of any type of breast cancer are also associated with Paget’s disease.
- A first-degree relative with breast or ovarian cancer
- Personal history of breast cancer, lobular carcinoma in situ (LCIS), dysplasia, or certain benign breast conditions
- Race: White women over 45 have a slightly higher risk of breast cancer than black and Hispanic women. For women under the age of 45, breast cancer is more common in African American women.
- Inherited gene mutation (for example, BRCA1 or BRCA2)
- dense breast tissue
- previous chest radiation exposure
- Use of postmenopausal hormone replacement therapy
- Being overweight, especially after menopause
- alcohol consumption
Causes and Risk Factors of Breast Cancer
Surgery is the main treatment option for Paget’s disease of the breast.
There are two options:
- Lumpectomy: This breast-conserving surgery removes the tumor from the breast. With Paget’s disease, the surgeon removes the nipple and areola, as well as the tapered portion of the breast. While ensuring that the cancer cells are removed, care is taken to preserve as much tissue as possible.
- Mastectomy: If the underlying breast cancer is invasive, a mastectomy (removal of the entire breast) will be performed. These will be removed if the lymph nodes are affected.
Radiation therapy is usually given after lumpectomy to kill any cancer cells that may be present in the remaining breast tissue. Many people have nipple reconstruction after mastectomy and radiation therapy.
The specific treatment recommended will depend on the extent of the cancer and the nature of the tumor (for example, if the tumor has estrogen or progesterone receptors).
how to treat breast cancer
After surgery, your healthcare provider may recommend other treatments to prevent breast cancer from coming back.
These treatments may include one or a combination of the following:
- Anticancer drugs (chemotherapy)
- hormone therapy
There are many risk factors beyond your control, such as getting older, but there are some lifestyle changes that may reduce your risk of breast cancer and Paget’s disease.
- Get regular physical activity
- avoid or limit alcohol consumption
- quit smoking
- maintain a healthy weight
Also, discuss breast cancer screening with your doctor so that if you develop breast cancer, it can be caught early. Get familiar with how your breasts usually look and feel and report any changes to your healthcare provider right away.
For high-risk patients
If you are at high risk for breast cancer, such as a family history or a genetic mutation that increases your risk, there are other steps you can take to try to prevent it and/or catch it early when it does occur. These options include:
- genetic counseling
- More frequent screening and self-exams to look for early signs of breast cancer
- Medications to reduce breast cancer risk
- prophylactic (preventive) surgery
Paget’s disease is a rare cancer that affects the nipple and areola of the breast. The disease mainly affects women in their 50s, but in rare cases it affects younger women and, in rare cases, men. People with Paget’s disease often also have invasive breast cancer. Risk factors include age, ethnicity, and genetic mutations. Surgery is a common treatment for Paget’s disease and its underlying cancer, sometimes along with chemotherapy, radiation therapy, and hormone therapy.
Because Paget’s disease is uncommon, you may know less about it than the more common types of breast cancer. Additionally, Paget’s support group is difficult to find in many communities due to the limited number of sick people. Fortunately, online communities allow you to connect with people around the world who share your diagnosis. Consider seeking support from others facing the same challenges.
Frequently Asked Questions
How fast does Paget’s disease progress?
Paget’s disease can develop over weeks to months. People may not experience symptoms right away, which can delay diagnosis.
Do you have Paget’s disease in both breasts?
This is unlikely. Paget’s disease usually occurs in one breast, but not both.
Will Paget’s disease of the breast show up on an ultrasound?
Paget’s disease itself doesn’t show up on an ultrasound, but because it’s often accompanied by an underlying breast cancer, the cancer is likely to show up on an ultrasound or a mammogram.
What is the prognosis for Paget’s disease?
The prognosis for Paget’s disease depends on the underlying breast cancer and whether it has spread to the lymph nodes. In one study, the mean 10-year survival rate for women with lymph node involvement was estimated to be 47%, while the mean 10-year survival rate for women without lymph node involvement was estimated to be 93%.
How can you tell the difference between Paget’s disease and eczema?
Paget’s disease usually affects one breast, while eczema may affect both. Paget’s disease does not respond to the medications you use to treat eczema.