Paget’s Disease of the Breast

Paget’s Disease of the Breast
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Updated on August 6, 2024
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Paget’s disease of the breast — also known as Paget’s disease of the nipple, mammary Paget’s disease, or sometimes Paget’s nipple disease — is a rare form of breast cancer that affects the skin of the nipple and usually the surrounding areola (the darker circle of skin around the nipple). It’s usually only found in one breast.

Paget’s disease occurs in about 1 to 3 percent of breast cancer cases, and is almost always found in the presence of other tumors in the same breast, including ductal carcinoma in situ (DCIS) or infiltrating ductal carcinoma (IDC).

Signs and Symptoms of Paget’s Disease of the Breast

The symptoms of Paget’s disease appear on the nipple and usually the areola. Most often, they occur on one breast, but they can also occur on both breasts. These symptoms include:

  • Itching or burning on the nipple
  • Crusting or oozing skin on the nipple or areola
  • Flaky or scaly skin on the nipple
  • Newly flattened or turned-in (inverted) nipple
  • A rash that can appear discolored, raised, or raw on the nipple area
  • Yellow or bloody nipple discharge

     
Paget’s disease is sometimes misdiagnosed as skin conditions such as breast eczema and dermatitis affecting the nipple, since the symptom can appear similar.

Complicating matters, the symptoms of Paget’s disease may appear to improve at first or respond to topical treatment, which can lead some people to think the skin is healing. Eventually though, the symptoms will worsen.

 Many people with Paget’s disease of the breast will go several months before they’re finally diagnosed.

Signs and Symptoms of Paget's Disease of the Breast, illustration of silhouetted woman center, fire icon for itching burning, crusting or oozing of nipple, yellow nipple discharge, flaking or scaling of nipple, newly flattened or inverted nipple, rash
The symptoms of Paget’s disease can appear similar to those of breast eczema and dermatitis affecting the nipple — which is one reason why the condition can be misdiagnosed.Everyday Health

Causes of Paget’s Disease of the Breast

It's not exactly known what causes Paget’s disease of the breast. The best theory researchers have so far is that cancer cells from a tumor in the breast move through the milk ducts to the nipple and areola. This is possibly why Paget’s is almost always found alongside other tumors in the same breast.

Another theory is that cells in the nipple or areola become cancerous on their own by developing genetic mutations — which may explain why some people with Paget’s develop the disease without having another type of breast cancer in the same breast.

Risk Factors of Paget’s Disease of the Breast

There are no known risk factors for Paget’s disease beyond those that can increase the risk of any kind of breast cancer. Some of the risk factors that are similar to those of any other type of breast cancer, according to the 2023 study in StatPearls, include:

  • A personal history of breast abnormalities, such as benign growths
  • A family history of breast or ovarian cancer
  • Dense breast tissue
  • Radiation exposure to the chest
  • Gene mutations, like BRCA1 and BRCA2
  • Hormone replacement after menopause

If you are at high risk of developing breast cancer from unavoidable factors like family history, your provider may recommend that you avoid or modify medical interventions like hormone replacement therapy and radiation exposure.

Diagnosis of Paget’s Disease of the Breast

Paget’s disease of the breast is diagnosed with a biopsy, in which a small area of the nipple is removed to be examined in a lab. Most likely, your doctor will use a circular cutting tool, called a punch, to remove a disk-shaped area of tissue. Sometimes, the entire nipple is removed.

If a biopsy identifies Paget’s disease of the breast, your doctor will also want to test for other breast cancers, including DCIS, using some of the following tests:

  • Breast exam, in which a doctor will check your breasts and lymph nodes for lumps or other abnormalities. About 50 percent of people with Paget’s disease of the breast have a breast lump that can be felt during a breast exam.

  • Mammogram, an X-ray of the breast tissue that is used to screen for breast cancer.
  • Breast ultrasound, a test that uses sound waves to create an image of the breast to screen for breast cancer.
  • Breast magnetic resonance imaging (MRI), which uses an MRI machine with a large magnet and radio waves to create pictures of the breast tissue and screen for breast cancer.
  • Breast biopsy, in which a small sample of breast tissue is removed and sent to a lab for analysis.
  • Sentinel lymph node biopsy, which may be done if imaging tests show invasive breast cancer. In this case, your doctor will want to see if the cancer has spread to the lymph nodes under your arms. If no cancer is found in a tissue sample from the sentinel node, it is unlikely the cancer has spread to any other nodes.

Treatment for Paget’s Disease of the Breast

If you don’t have any other type of breast cancer, your doctor may recommend treating Paget’s disease with breast-conserving surgery (lumpectomy), a procedure that removes the cancer while leaving as much healthy breast tissue as possible. For Paget’s disease, the nipple and the areola are removed.

If Paget’s disease is detected alongside other tumors of the breast, your doctor may recommend more extensive surgery, such as a mastectomy, a procedure which removes the entire breast, and lymph node removal surgery, in which the lymph nodes are removed. After a mastectomy, you may choose to have breast reconstruction surgery.

After the surgery, you may also need to have other treatments, called adjuvant therapy, to prevent the cancer from recurring. This may include radiation therapy, chemotherapy, and hormone therapy.

Paget’s Disease Prognosis

If Paget’s disease hasn’t spread to the surrounding breast tissue, the prognosis is excellent.

 According to the NCI’s Surveillance, Epidemiology, and Ends Results (SEER) program, the overall five-year survival rate for women with Paget’s was 82.6 percent.
The five-year survival rate tends to decrease as the stage of the cancer increases; for women with stage 1 Paget’s disease, the survival rate is 95.8 percent; stage 2 is 77.7 percent; stage 3 is 46.3 percent; stage 4 is 14.3 percent.

Prevention of Paget’s Disease of the Breast

There’s no known way to prevent Paget’s disease, but you can reduce your risk for breast cancer in general by making some of the following lifestyle modifications.

  • Avoid or limit your alcohol intake. Alcohol can increase the risk of breast cancer, so try to limit yourself to no more than one alcoholic drink a day.
  • Maintain a healthy weight. Overweight and obesity can increase the risk of breast cancer.
  • Exercise regularly. Try to get at least 150 to 300 minutes of moderately intense physical activity each week, or 75 to 150 minutes of vigorously intense physical activity per week.
  • Limit postmenopausal hormone therapy. Hormone therapy to ease menopause symptoms can increase the risk of breast cancer, so talk to your doctors about nonhormonal options or limiting the dosage of hormone therapy.
  • Ask about genetic testing. If you suspect you may have a gene that increases your risk of breast cancer — for example, you have a strong family history of the disease — talk to your doctor about doing genetic testing or genetic counseling.

The Takeaway

Paget’s disease of the breast is a rare form of breast cancer that occurs in the nipple and usually the areola. It’s oftentimes found alongside other forms of breast cancer, such as DCIS. If Paget’s disease hasn’t spread to other areas of the breast, it can likely be treated with surgery such as a lumpectomy, which can preserve some of the original breast tissue.

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Ryland J. Gore, MD, MPH

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Ryland Gore, MD, MPH, is a board-certified, fellowship-trained surgeon specializing in breast surgical oncology in Atlanta. She completed her general surgery residency at Rush University Medical Center and John H. Stroger Cook County Hospital in Chicago. She went on to complete her breast surgical oncology fellowship at Maimonides Medical Center in Brooklyn, New York.

In addition to her professional responsibilities, Gore previously served on the board of directors for Every Woman Works, an Atlanta-based nonprofit organization whose mission is to empower women and help them transition into independence and stability from common setbacks. Gore served as the chairwoman of the American Cancer Society’s Making Strides Against Breast Cancer campaign in Atlanta for three years (2019 to 2021). She is currently the co-director of Nth Dimensions’ Strategic Mentoring Program and the alumni board chair of the Summer Health Professions Educational Program (SHPEP), which is a collaborative effort by the Robert Wood Johnson Foundation, Association of American Medical Colleges, and the American Dental Education Association.

Gore is a highly sought after speaker, consultant, and lecturer on breast cancer and breast health, as well as women’s empowerment topics.
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Cathy Cassata

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Cathy Cassata is a freelance writer who specializes in stories about health, mental health, medical news, and inspirational people. She writes with empathy and accuracy, and has a knack for connecting with readers in an insightful and engaging way. Cassata contributes to Healthline, Verywell, Yahoo, and more.

She previously worked for the American Association of Medical Assistants for eight years, writing and editing the magazine, marketing materials, and the website. Cassata completed the editing certificate program at the University of Chicago.

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Resources
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  5. Yasir M et al. Mammary Paget Disease. StatPearls. June 3, 2023.
  6. Paget’s Disease of the Breast. Cleveland Clinic. November 2, 2022.
  7. Markarian S et al. Mammary Paget's Disease: An Update. Cancers. May 23, 2022.
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  9. Paget's Disease of the Breast. National Cancer Institute. April 10, 2012.
  10. Can I Lower My Risk of Breast Cancer? American Cancer Society. December 16, 2021.