An Overview of Paget's Disease of the Breast

A rare type of breast cancer affecting the nipple or areola

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Paget's disease, a type of breast cancer
Paget's Disease. IAN HOOTON / Getty Images

Paget’s disease of the breast-sometimes called Paget’s disease of the nipple is a rare type of breast cancer. Paget’s starts on a nipple and extends to the areola (dark circular part around the nipple). Paget’s cells are usually found the surface of the nipple and areola. Paget’s disease of the breast does not cause breast lumps or tumors. This breast condition usually affects women over age 50, but it can affect women and men of any age.

Paget's disease was named after Sir James Paget, a British doctor from the 1800s who published articles about the link between changes to the nipple and underlying breast cancer. Paget’s accounts for 5 percent of all breast cancers. Paget's most commonly affects people in their 50s, especially menopausal women. That said, the disease may also appear in adolescents and the elderly. Male breast cancers only account for 0.5 percent of breast cancers and Paget’s in men is even rarer.  

Is There Always Another Underlying Cancer? 

In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. In these cases, nipple skin cells may spontaneously change into cancer cells.

However, the majority of people with Paget's disease of the nipple have either invasive breast cancer or ductal carcinoma in situ (DCIS). Researchers believe cancer cells may travel from the tumor and float up through the milk ducts where they enter the nipple and areola. Breast tumors are almost always found in the same breast that has Paget's disease cells. 

Signs and 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 will come and go.

Symptoms will worsen with time and may include:

  • Itching or a burning sensation in the nipple and/or areola
  • Pain and sensitivity
  • Flattening of the nipple
  • Yellow or bloody discharge from the nipple
  • A lump in the affected area

Symptoms of Paget’s disease are sometimes mistaken for symptoms of skin conditions, including eczema or dermatitis. Paget's is usually only found in one breast and does not respond to topical medications.

If you have any of the signs or symptoms noted above, see you doctor as soon as possible. 

Causes and Risk Factors

Doctors does know exactly what causes Paget’s disease of the breast. Most researchers think the disease is the result of an underlying type of ductal breast cancer. Another theory is that Paget’s cells develop independently.

Risk factors that increase the result for any type of breast cancer are also associated with Paget’s disease. These include:

  • Age: Your chances of developing breast cancer increase as you get older.
  • Race: White women have a higher risk for breast cancer than do black and Hispanic women. But black women are more likely to die from breast cancer.
  • Personal or family history of breast cancer: Previous history of breast cancer in one breast increases risk for Paget’s disease.  If you have had lobular carcinoma in situ or atypical hyperplasia, your risk for breast cancer is higher. Some benign breast conditions also increase risk. If your mother, sister, or daughter previously had breast or ovarian cancer, you have a higher risk for breast cancer.
  • Inherited gene mutation: Certain gene defects, including BRCA1 and BRCA2 increase risk for breast cancer and other cancers. 
  • Dense breast tissue: Some women have dense breast tissue, which can be seen mammograms. Dense tissues increase risk for both Paget’s and breast cancer.
  • Previous radiation exposure: If you have received radiation treatment to your chest to treat another type of cancer in the past, you have a higher risk for Paget’s disease.
  • Hormone replacement: Taking estrogen hormone therapy after menopause can increase risk.
  • Excess weight: Being overweight especially after menopause is a risk factor for breast cancer and Paget’s.
  • AlcoholExcessive alcohol consumption increases breast cancer risk.

Having certain risk factors does not mean a person will be diagnosed with Paget’s or another form of breast cancer. In fact, most women who develop breast cancer usually have no known risks. 

Diagnosis

If you are experiencing any of the above signs and symptoms, see a health professional. Diagnosing Paget's disease will include the following steps: 

  • Clinical breast exam: Up to 50 percent 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: In Paget’s disease of the breast, a mammogram may reveal nipple and skin changes that are linked to underlying breast cancer. If a mammography of both breasts don’t show anything unusual or any signs of cancer, your doctor may follow up with magnetic resonance imaging (MRI), which may detect cancer that cannot be seen on a mammogram.
  • Breast biopsy: During a breast biopsy, a small sample of tissue is taken from the skin of the nipple for examination under a microscope. If there is nipple discharge or a breast lump, discharge and tissue samples from the lump will also be taken.  If cancer cells are detected in any of the samples, your doctor will refer you to a surgeon to discuss treatment.
  • Sentinel lymph node biopsy: During a sentinel lymph node biopsy, the surgeon locates the sentinel nodes, as these are the first place breast cancer cells will go to. If a sentinel node is removed, examined and found normal, it means finding cancer in remaining nodes is unlikely and no other nodes will be removed or examined. 

    Treatment

    The most common treatments for Paget's disease of the breast are surgery, chemotherapy, radiation, and hormone therapy.

    Surgery

    • Mastectomy. A mastectomy surgery involves removing the entire breast, but not the lymph nodes in the armpit. A mastectomy is needed if the underlying breast cancer is invasive and removing the entire breast makes more sense. Removing the entire breast means that the nipple and areola are also removed.  If lymph nodes are affected, these will be removed with a sentinel lymph node biopsy.
    • Lumpectomy: An lumpectomy is a breast-conserving surgery that involves removal of only the diseased portion of the breast. With Paget’s disease, the surgeon removes the nipple and areola along with a cone-shaped part of the breast. The surgeon will remove as little tissue as possible, while making sure the cancer cells are removed. If a lumpectomy surgery is used to treat Paget’s disease of the breast, it is followed with radiation therapy. Many women will have nipple reconstruction after their lumpectomy and once radiation is completed. 

    Additional Treatments

    After surgery, your doctor may recommend additional treatment with anti-cancer drugs (chemotherapy), radiation, or hormone therapy to prevent a breast cancer recurrence. The specific treatment will depend on the extent of the cancer and the nature of the tumor, i.e. if the tumor has estrogen or progesterone receptors.

    A Word From Verywell

    Since Paget's disease is an uncommon type of breast cancer, you've likely heard less about this disease than the most common forms. It can also be difficult to find a support group in many communities due to the limited number of people with the disease. Fortunately, online communities now allow people to connect with others around the globe so that people living with Paget's disease can find support from others facing the same challenges.

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