Paget's Disease of the Breast

What Is Paget's Disease of the Breast?

Paget's disease of the breast is an uncommon type of cancer that forms in or around the nipple. It is involved in 1% to 4% of all breast cancers. It may be linked with an underlying breast cancer, either ductal carcinoma in situ or invasive breast cancer. You might hear it called Paget’s disease of the nipple or mammary Paget disease.

Who Gets Paget’s Disease of the Breast?

Paget’s disease of the breast can affect men and women of any age, but women are more likely to get it. The average age at diagnosis is 57. A small number of people with breast cancer will get it, too.

What Are the Symptoms of Paget's Disease of the Breast?

Early symptoms include redness and light scaling and flaking of the skin on your nipple. You might only notice mild irritation -- not enough to see the doctor. Your skin could get better on its own, but this doesn’t mean the disease has disappeared.

More advanced symptoms reflect serious damage to your skin. You might also notice:

  • Tingling
  • Itching
  • Increased sensitivity
  • Burning
  • Pain
  • Bloody or yellowish discharge from your nipple
  • Your nipple looks flattened against your breast
  • Thickened skin in the nipple area

In most cases, Paget's disease of the breast starts in the nipple, then spreads to the areola (the circular area of darker skin that surrounds it) or other areas of your breast. Sometimes it affects only the areola, where it can look like eczema, an itchy red rash. It’s rare, but this condition can affect both breasts.

What Causes Paget’s Disease of the Breast?

Doctors aren’t sure what causes it. Though Paget’s is rare, most people who get it have tumors in the same breast. One theory is that cancer cells from the tumor travel through milk ducts and into your nipple and areola.

Risk Factors for Paget’s Disease

Things that can make you more likely to get Paget’s disease of the breast include:

  • Age: Your chances rise as you get older.
  • Alcohol: Drinking heavily makes it more likely.
  • Dense breasts: This will show up on a mammogram.
  • Extra weight: This is especially true after menopause or if you gained weight as an adult.
  • Family history: Your chances go up if your parents or siblings -- male or female -- had breast cancer.
  • Gene defects: You might hear them called mutations. BRCA1 and BRCA2 make you more likely to get breast and ovarian cancers, but they only lead to fewer than 1 out of 10 breast cancers.
  • History of breast cancer: If you’ve had it in either breast, your chances are greater.
  • History of breast abnormalities: Having lobular carcinoma in situ or atypical hyperplasia raises your odds.
  • Hormone replacement: Taking estrogen after menopause makes it more likely.
  • Race: White women are more likely to get breast cancer than black or Hispanic women, but black women are most likely to die from it.
  • Radiation: If you got radiation to your chest as a child or young adult, you’re more likely to get breast cancer.

Continued

How Is Paget's Disease of the Breast Diagnosed?

If your doctor thinks you might have it, they’ll do a test called a biopsy. The doctor will remove a small sample of tissue from your nipple and send it to a lab where they’ll check for the presence of Paget's cells.

Because most people with this condition also have underlying breast cancer, your diagnosis will probably include:

  • A physical exam
  • Imaging tests:
    • A mammogram (X-ray of the breast)
    • Breast MRI (a scan that creates a detailed picture of the inside of your breast)
    • Breast ultrasound (uses sound waves to make a picture of your breast)

How Is Paget's Disease of the Breast Treated?

Surgery is the most common treatment. You might have:

  • Mastectomy. The surgeon removes the entire breast.
  • Lumpectomy and radiation. If your disease is confined to the nipple and the surrounding area, your doctor could suggest this treatment to save your breast (you might hear it called breast-conserving surgery). The surgeon will remove your nipple, areola, and the entire portion of the breast believed to contain the cancer. Most women get follow-up radiation therapy to make sure the cancer doesn’t come back.

The specific treatment often depends on the underlying breast cancer.

What’s the Outlook for Paget's Disease of the Breast?

The outlook depends on:

  • If you have a tumor in the affected breast
  • If the tumor is ductal carcinoma in situ or invasive breast cancer
  • The stage of invasive breast cancer in that breast
  • If you have invasive cancer in the affected breast and it has spread to nearby lymph nodes

The 5-year relative survival for all women in the U.S. diagnosed with this condition from 1988 to 2001 was 82.6%. This compares with a 5-year relative survival of 87.1% for women diagnosed with any type of breast cancer in that same time.

But for women with both Paget's disease of the breast and invasive cancer in the same breast, the 5-year relative survival declined with increasing stage of the cancer:

  • Stage I: 95.8%
  • Stage II: 77.7%
  • Stage III: 46.3%
  • Stage IV: 14.3%

WebMD Medical Reference Reviewed by Michael W. Smith, MD on August 26, 2019

Sources

SOURCES: 

Kaelin CM. Paget’s Disease. In: Harris JR, Lippman ME, Morrow M, Osborne CK, editors. Diseases of the Breast. 3rd ed. Philadelphia: Lippincott Williams and Wilkins, 2004.

DeVita,VT Jr., Hellman S, Rosenberg SA, editors. Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia: Lippincott Williams and Wilkins, 2004.

Beers MH, Berkow R, editors. The Merck Manual of Diagnosis and Therapy. 17th ed. Whitehouse Station, NJ: Merck & Company, Inc., 1999.

American Cancer Society (2005). Cancer Facts and Figures 2005. Atlanta, GA: American Cancer Society. Retrieved April 20, 2005, from http://www.cancer.org/downloads/STT/CAFF2005f4PWSecured.pdf.

Marcus E. The management of Paget’s disease of the breast. Current Treatment Options in Oncology 2004; 5:153-160.

National Cancer Institute: “Paget Disease of the Breast.”

© 2019 WebMD, LLC. All rights reserved.

Pagination