Paget's Disease of the Breast

What Is Paget's Disease of the Breast?

 

Paget's disease of the breast is a rare type of cancer that forms in or around your nipple. It’s 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 also hear it called Paget’s disease of the nipple or mammary Paget disease.

Who Gets Paget’s Disease of the Breast?

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

  • Age. Your chances go up as you get older. The average age at diagnosis is 57.
  • Sex. Paget’s can affect men and women, but women are more likely to get it.
  • 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.
  • Alcohol . Drinking heavily makes it more likely.
  • Dense breasts . This will show up on a scan called 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 had breast cancer.
  • Gene changes. You might hear them called mutations. BRCA1 and BRCA2 make you more likely to get breast and ovarian cancers, but they 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 higher.
  • History of unusual things in your breast (abnormalities). Having lobular carcinoma in situ or atypical hyperplasia raises your odds.
  • Hormone replacement . Taking estrogen after menopause makes cancer more likely.
  • Radiation . If you got radiation to your chest as a child or young adult, you’re more likely to get breast cancer.

Paget's Disease of the Breast Symptoms

Early symptoms include redness, light scaling, and flaking of the skin on your nipple. You might have only mild irritation, not enough to send you to the doctor. Your skin could get better on its own. This doesn’t mean the disease has gone away.

Continued

Further symptoms can be a sign of serious damage to your skin. You might notice:

  • Tingling
  • Itching
  • More sensitivity
  • Burning
  • Pain
  • Bloody or yellowish discharge from your nipple
  • Oozing or bleeding areas
  • A nipple that looks flattened against your breast
  • Thickened skin in the nipple area
  • A lump or mass in your breast

In most cases, the disease starts in the nipple and 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.

Rarely, this condition can affect both breasts.

Paget’s Disease of the Breast Causes

Doctors aren’t sure what causes it. Paget’s is rare, but 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.

Paget's Disease of the Breast Diagnosis

Your doctor will do a test called a biopsy. They’ll take a small sample of tissue from your nipple and send it to a lab, where a technician will check for Paget's cells. Your doctor will take the sample in one of a few ways:

  • Surface biopsy. They’ll gently scrape cells from the surface of your skin.
  • Shave biopsy. They’ll use a very sharp tool to take off the top layer of skin.
  • Punch biopsy. A special circular tool called a punch takes a small, round piece of tissue.
  • Wedge biopsy. Your doctor uses a scalpel to take a small chunk of tissue.
  • In some cases, the doctor removes the entire nipple.

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

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

Continued

Paget's Disease of the Breast Treatment

The treatment depends on the underlying breast cancer. You might have one or more these:

  • Mastectomy . Your surgeon removes your entire breast.
  • Lumpectomy . If the disease affects only your nipple and the area around it, your doctor could suggest this treatment to save your breast. They might call it breast-conserving surgery. The surgeon will remove your nipple, your areola, and the part of the breast where they think the cancer is.
  • Radiation. High-energy radiation can kill cancer cells. It might come from a machine outside your body (external) or from a small implant in your breast (internal).
  • Chemotherapy. Certain drugs also kill cancer cells. You can get them in a pill that you swallow or in a liquid that goes into your vein (intravenous).
  • Hormonal therapy. These drugs keep estrogen from reaching cancer cells so cancer doesn’t grow or come back. They work on cancers that are positive on a hormone receptor (HR) test. You could get a selective estrogen receptor modulator (SERM) or an aromatase inhibitor (AI). They come in pills.

Paget’s Disease of the Breast Complications

Cancer treatment can have side effects that last a long time or that start weeks, months, or years after you finish treatment. These include:

  • Pain and numbness (peripheral neuropathy)
  • Swelling in your arms or legs (lymphedema)
  • Bone loss (osteoporosis)
  • Infertility
  • Heart problems
  • Blood clots
  • New cancer

Paget’s Disease of the Breast Prevention

There’s no definite way to prevent cancer, but some lifestyle changes can help make it less likely.

  • Keep a healthy weight.
  • Get regular exercise. Moderate to high-intensity activity is best.
  • Limit how much alcohol you drink. Experts recommend no more than one drink a day for women and two for men.
  • Breastfeeding may also lower your risk of breast cancer.

If you have one or more breast cancer risk factors, talk to your doctor about further steps, including:

  • Testing to look for changes in your genes
  • More frequent checkups or screening tests
  • Medicines to block estrogen such as raloxifene and tamoxifen
  • Breast removal surgery (prophylactic mastectomy)

Continued

Paget's Disease of the Breast Outlook

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’s spread to nearby lymph nodes

About 83% of women in the U.S. diagnosed with this condition from 1988 to 2001 lived at least 5 more years. By comparison, about 87% of women diagnosed with any kind of breast cancer in that period lived at least 5 more years.

For women with both Paget's disease of the breast and invasive cancer in the same breast, the 5-year relative survival rate went down with each 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 Gabriela Pichardo on May 15, 2020

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 https://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.”

National Organization for Rare Disorders: “Paget’s Disease of the Breast.”

Cedars-Sinai: “Paget’s Disease of the Breast.”

Johns Hopkins Medicine: “Adjuvant Hormonal Therapy,” “Side Effects From Breast Cancer Treatment.”

American Cancer Society: “Can I Lower My Risk of Breast Cancer?”

© 2020 WebMD, LLC. All rights reserved.

Pagination