Paget disease of the breast is a rare type of breast cancer. It affects the nipple and its surrounding tissues. Symptoms may include nipple discharge, itching, soreness, or changes in skin coloring. Treatment is available, and the outlook is positive.
Medically reviewed by Teresa Hagan Thomas PHD, BA, RN
Medically reviewed by Teresa Hagan Thomas, PhD, BA, RN on May 9, 2023
Paget or Paget’s disease of the breast is a form of breast cancer that affects the nipple and areola.
Paget’s disease may affect other tissues in the body. Different types include Paget’s disease of the:
bone
penis
vulva
These are all rare forms of cancer, but they are not related to each other.
This article will overview Paget’s disease of the nipple, its symptoms and causes, and treatment options.
What is Paget’s disease of the breast?
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Sometimes known as Paget’s disease of the nipple or mammary Paget disease, the condition is a rare form of cancer that affects mammary tissues. It usually develops in one breast only.
Paget’s disease of the breast was named after a British doctor called Sir James Paget, who first noticed a connection between breast cancer and changes in the nipple.
Paget’s disease of the breast is considered rare as only 1% to 4% of breast cancer cases involve Paget’s disease.
Mammary Paget disease may affect both males and females, although females are more likely to develop it. Although people of all ages may have it, the average age of diagnosis for Paget’s disease of the breast is 57.
You may have a greater chance of developing Paget’s disease of the breast if you:
These are also contributing factors to other types of breast cancer.
Causes and contributing factors
The cause of Paget’s disease of the breast has not been established yet, but two main theories have been put forward. One theory suggests that the cells in the areola or nipple become cancerous on their own, as it happens in other tissues.
The second theory suggests that Paget’s disease of the breast is caused by cancer cells migrating from an existing tumor in other areas. The Genetic and Rare Diseases Information Center (GARD) and other experts suggest the condition may result from cancer cells traveling through the mammary milk ducts from a tumor near the nipple and areola tissues.
Symptoms
The symptoms of Paget’s disease of the breast can be different from other forms of breast cancer. With this condition, you will likely first notice changes in your nipple or areola.
The symptoms of Paget’s disease of the breast may include:
tingling, itching, or changes in skin color in the areola or nipple
flattening of the nipple
thickened, flaky, or crusty skin on or near the nipple
bloody or yellow discharge from the nipple
increased sensitivity, soreness, and pain in the breast tissues
People with the condition may have one or more breast tumors in the same tissues where the symptoms of Paget’s disease are visible. But not all people with Paget’s disease of the breast have tumors.
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Paget’s disease of the breast often begins with changes in the skin of the nipple or areola. Some of these symptoms resemble an inflammatory skin condition known as eczema.
Itching, changes in skin color, flaking, and dry skin are some of the symptoms eczema shares with Paget’s disease of the breast. Still, unlike eczema, Paget’s disease of the breast usually affects one side only.
Topical creams and treatments commonly used for eczema will not be effective in treating skin symptoms of Paget’s disease of the breast.
Only a healthcare professional may be able to diagnose these conditions accurately. If you notice changes to your nipples or the skin around them, they can help you explore possible causes.
Diagnosis
To diagnose Paget’s disease of the breast, a medical professional may first carry out a physical examination of your breasts and nipples. They may also suggest a mammogram to check for other signs of breast cancer. An MRI scan or ultrasound could follow this if they notice any growths or changes in the tissue.
A diagnosis of Paget’s disease of the breast is usually confirmed with a biopsy, though. This involves a small sample of the affected tissue in the nipple, areola, or growth. This tissue is then taken to a laboratory where experts will look for the presence of cancerous cells.
Treatment
Paget’s disease of the breast is typically treated by a cancer specialist, also known as an oncologist. They will explore possible treatment plans with you.
What treatment you receive may depend on the affected tissue and how advanced the condition is. This includes determining whether the cancer has spread to other organs or tissues, the grade and size of tumors, and your overall health.
Your treatment options may involve one or more strategies, including:
Breast-conserving surgery: This surgery removes the areola and nipple without touching the rest of the breast. This treatment is likely if you don’t have breast tumors.
Surgery to remove your breast (mastectomy): This may be recommended for some people with breast tumors.
Lymph node removal surgery: The surgery aims to remove affected lymph nodes if the cancer has spread to these tissues.
Chemotherapy: This therapy works with medications that target cancer cells, making them more vulnerable to the immune system defenses.
Radiation therapy: It may be used on its own, but it is often combined with chemotherapy. This therapy uses radiation to kill cancer cells or shrink cancerous growths directly.
Additional targeted therapies: Depending on your case, your healthcare team may use other targeted treatments, particularly if you have HR-positive tumors.
Outlook
Many factors affect how Paget’s disease of the breast may respond to treatment. These include:
overall health
age
individual response to chosen treatments
existence of tumors underneath the nipple, and their grade and stage
early versus later diagnosis
According to existing data, Paget’s disease of the breast has an overall positive outlook, with 5-year survival rates between 63% and 90%, depending on how advanced the disease is at the time of treatment.
Still, because cancer outlook varies from person to person, you may want to discuss your particular case with your healthcare team.
Summary
Paget’s disease of the breast is a rare form of breast cancer that affects the nipple and areola tissues in both males and females.
Symptoms may include nipple discharge, itchiness, skin texture, color changes, and soreness in the area.
Early stages of Paget’s disease of the breast may be misdiagnosed as eczema and other skin conditions.
Only a healthcare professional can explore possible causes of your symptoms.
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