An Overview of Paget's Disease of the Breast

Paget's disease of the breast is a rare type of cancer of the nipple and areola that is often associated with a type of invasive cancer called ductal carcinoma in situ.

Paget’s disease occurs in approximately 1% to 4% of all breast cancers. It most commonly affects women between the ages of 40 and 60, especially during menopause. This disease may also appear in adolescents and older people, and in rare cases can occur in men.

Several unrelated diseases are also named after Paget, including a skeletal condition known as Paget's disease of the bone and extramammary Paget disease, which includes Paget disease of the vulva and Paget disease of the penis. This article only covers Paget's disease of the breast.

This article will discuss symptoms, causes, treatment options, and when to see a healthcare provider.

Symptoms

Paget’s disease causes the skin on the nipple and the area around it to be red, sore, flaky, and scaly. Early on, these symptoms may come and go.

Symptoms usually worsen with time and may include:

• Itching or a burning sensation in the nipple and/or areola
• Flaking, crusty, or thickened skin on or around the nipple
• Pain and sensitivity
• Flattening of the nipple
• Yellow or bloody discharge from the nipple
• A lump in the affected area

When to See a Healthcare Provider

If you are experiencing any signs and symptoms of Paget's disease, see a healthcare provider as soon as possible. Early diagnosis increases the chances of successful treatment.

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 have a breast lump or an area of thickening that is felt upon clinical exam.
• Mammography: If you have Paget’s disease of the breast, a mammogram may show nipple and skin changes that are linked to underlying breast cancer. Your healthcare provider may also follow up with magnetic resonance imaging (MRI).
• Breast biopsy: During a breast biopsy, a small sample of tissue is taken from the lump, skin, or nipple for microscopic examination. If there is nipple discharge, it will be collected and examined as well.
• Sentinel lymph node biopsy: During this procedure, a surgeon locates and removes sentinel nodes—the first place breast cancer cells go. If a sentinel node is examined and found to be negative, it is unlikely that cancer will be found in the remaining nodes.

If cancer cells are detected in any of the biopsy samples, you will need treatment.

Causes

The cause of Paget’s disease of the breast is not known. About 80% to 90% of people with Paget's also have DCIS or invasive cancer somewhere else in the breast, according to the American Cancer Society.

Cancer cells may travel from the tumor up through the milk ducts, where they enter the nipple and areola.

Sometimes 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 these instances, nipple skin cells may spontaneously change into cancer cells.

Risk Factors

The same factors that increase the risk of any type of breast cancer are also associated with Paget’s disease. 

These include:

• Increasing age
• First degree relative with breast or ovarian cancer
• Personal history of breast cancer, lobular carcinoma in situ (LCIS), atypical hyperplasia, or certain benign breast conditions
• Race: White women who are over 45 have a slightly higher risk of breast cancer than Black and Hispanic women. For women under age 45, breast cancer is more common in African American women. 
• Inherited gene mutation (e.g., BRCA1 or BRCA2)
• Dense breast tissue
• Previous radiation exposure to the chest
• Use of hormone replacement therapy after menopause
• Being overweight, especially after menopause
• Alcohol consumption

Treatment

Surgery is the primary 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, along with a cone-shaped part of the breast. Care is taken to spare as much tissue as possible while ensuring that the cancer cells are removed.
• Mastectomy: If the underlying breast cancer is invasive, a mastectomy (removal of the whole breast) will be done. If lymph nodes are affected, these will be removed.

Lumpectomy is usually followed by radiation therapy to kill any cancer cells that may be in the remaining breast tissue. Many people will have nipple reconstruction after lumpectomy and radiation are completed. 

The specific treatment that's 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).

Adjuvant Therapy

After surgery, your healthcare provider may recommend additional treatments to try to prevent a breast cancer recurrence.

These therapies may include one or a combination of the following:

• Anti-cancer drugs (chemotherapy)
• Hormone therapy
• Radiation

Prevention

There are many risk factors that you can't control, such as getting older, but there are some lifestyle modifications that may reduce your risk of breast cancer and Paget's disease.

These include:

• Get regular physical activity
• Avoid or limit alcohol
• Quit smoking
• Maintain a healthy weight

In addition, discuss breast cancer screenings with your doctor so that if you develop breast cancer, it can be detected as early as possible. Be familiar with how your breasts normally look and feel and report any changes to a healthcare provider right away.

For High-Risk Patients

If you have a high risk of breast cancer, such as a family history or a gene mutation that raises your risk, there are additional steps you can take to try to prevent it and/or catch it early if it does occur. These options include:

• Genetic counseling
• More frequent screenings and self-checks to look for early signs of breast cancer
• Medicines to lower breast cancer risk
• Preventive (prophylactic) surgery

Summary

Paget's disease is a rare form of cancer that affects the nipple and areola of the breast. This disease affects mostly women in their 50's but in rare cases can affect younger women and in very rare cases, men. People with Paget's disease usually also have invasive breast cancer. Risk factors include age, race, and inherited mutations. Surgery is the usual treatment for Paget's disease and its underlying cancer, sometimes along with chemotherapy, radiation, and hormone therapy.

A Word From Verywell

Since Paget's disease is not common, you've likely heard less about it than more common types of breast cancer. In addition, it can be difficult to find a Paget's support group in many communities due to the limited number of people with the disease. Fortunately, online communities give you the ability to connect with people around the world who share your diagnosis. Consider reaching out for support from others facing your same challenges.