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Paget Disease of the Breast

Paget disease of the breast, also called Paget disease of the nipple and mammary Paget disease, is a rare type of breast cancer that affects the skin of the nipple and the areola, the circle of darker skin around the nipple.
 

Paget disease of the breast, also called Paget disease of the nipple and mammary Paget disease, is a rare type of breast cancer that affects the skin of the nipple and the areola, the circle of darker skin around the nipple.

In Paget disease of the breast, cancerous cells called Paget cells are found in the surface layer of the skin of the nipple and the areola. Most people diagnosed with Paget disease of the breast have one or more tumors in the same breast.

About 1-4% of all breast cancer cases also involve Paget disease of the breast.

Doctors aren’t sure what causes Paget disease of the breast. The theory many doctors accept is that cancer cells from a tumor in the breast travel through the milk ducts to the nipple and areola. This could explain why Paget disease of the breast and one or more tumors in the same breast are almost always found together.

It could also be that cells in the nipple or areola turn cancerous by themselves. This could explain why some people develop Paget disease of the breast and have no tumor in the same breast. It also may be possible that Paget disease of the breast and a tumor in the breast develop independently of each other.

 

Symptoms of Paget disease of the breast

The symptoms of Paget disease of the breast are similar to those of some non-cancerous conditions, such as dermatitis or eczema, and include:

  • itching, tingling, or redness in the nipple or areola area

  • flaking, crusty, or thickened skin on or around the nipple

  • flatting of the nipple

  • yellowish or bloody nipple discharge

 

Diagnosis of Paget disease of the breast

Diagnosing Paget disease of the breast usually involves a combination of procedures and almost always includes:

 

Treatment of Paget disease of the breast

Treatments for Paget disease of the breast usually include:

If there is a tumor in the same breast, it will be staged and treated like invasive ductal carcinoma.

 

Survivorship care after Paget disease of the breast treatment

Because of treatments they’ve received, many breast cancer survivors have a higher risk of developing other diseases as they age, including high blood pressure, heart disease, and osteoporosis. To make sure breast cancer survivors are regularly screened for these and other diseases, experts have developed the idea of survivorship care planning.

Survivorship care plans are written documents made up of two parts.

The first part is a treatment summary, a record of all the breast cancer treatments you’ve received.

The second part is basically a roadmap of what you can expect in the years after treatment, including any late or long-term side effects you might have, and a schedule of how you’ll be monitored for these side effects and other health conditions. This part of the survivorship care plan usually includes:

  • the tests you’ll have

  • which doctors will order the tests

  • a schedule of when the tests will be done

  • healthy living recommendations

  • resources, if you need more information

Learn more at Before Treatment: Planning Ahead for Survivorship.

Reviewed by 1 medical adviser
 
Jenni Sheng, MD
Johns Hopkins University School of Medicine, Baltimore, MD
Learn more about our advisory board

— Last updated on July 26, 2022, 5:58 PM