Paget’s Disease of the Breast

Overview

What is Paget’s disease of the breast?

Paget’s disease of the breast is cancer that forms in the nipple or areola (darker skin surrounding it). This rare condition usually occurs along with underlying cancer in the same breast.

Weepy, irritated skin on the nipple is often the first sign of this rare cancer although most people with nipple changes do not have Paget’s disease. It is also known as mammary Paget disease or Paget’s disease of the nipple.

How common is Paget’s disease of the breast?

Paget’s disease makes up about 1-4% of breast cancers. It develops in both women and men, but happens more in women. It most commonly occurs in women over 50 years old.

Symptoms and Causes

What causes Paget’s disease of the breast?

Doctors are not certain what causes Paget’s disease of the breast. Many doctors believe it forms when underlying cancer in the same breast makes its way to the nipple via the milk ducts.

What are the symptoms of Paget’s disease of the breast?

For people with Paget’s disease of the breast, symptoms in the nipple are often the first indication that cancer has developed in the breast.

The signs of Paget’s disease of the breast resemble common skin conditions including eczema (very dry, scaly skin) and dermatitis (a type of eczema that shows up as an itchy rash). Signs of Paget’s disease of the breast include:

  • Crusty and red skin of the nipple or the areola
  • Burning or itching of the skin of or near the nipple
  • Flat or inverted (turned-in) nipple
  • Bloody or yellow discharge from the nipple

Diagnosis and Tests

How is Paget’s disease of the breast diagnosed?

A doctor diagnoses Paget’s disease of the breast with a test called a biopsy. During this test, a doctor removes a small piece of breast tissue and looks at it under a microscope. In some cases, the doctor removes the entire nipple for testing.

Your doctor may also perform a physical breast exam and imaging tests such as a mammogram, an ultrasound, or magnetic resonance imaging scan (MRI). These tests enable the doctor to check for other signs of cancer such as a lump or other breast changes.

Management and Treatment

How is Paget’s disease of the breast treated?

Treatment for Paget’s disease of the breast usually begins with surgery to remove the cancer.

Your doctor may remove the entire breast in an operation called a mastectomy. Doctors typically use this treatment when there is also breast cancer in other areas of the breast.

People who have a mastectomy may also have a sentinel lymph node biopsy (removal and examination of the lymph nodes where the cancer is most likely to spread). This test tells the doctor if the cancer has spread to other lymph nodes in the body.

In some cases, doctors remove only the area of the breast that contains cancer. This operation is called breast-conserving surgery.

What other treatments are common after surgery for Paget’s disease of the breast?

Many people who have surgery for Paget’s disease of the breast have radiation therapy after the operation. Radiation delivers strong X-rays to the breast to destroy any remaining cancer cells.

Some people with Paget’s disease of the breast receive chemotherapy. This treatment delivers medication throughout the body to destroy any cancer cells left after surgery. Doctors deliver this medicine into the vein through an IV or with a pill.

Sometimes doctors recommend hormone therapy for people diagnosed with hormone receptor-positive breast cancer. This type of cancer grows because of hormones naturally produced in the body. Treatment involves lowering the levels of hormones in the body or blocking them from the tumor so it cannot grow.

What complications are associated with Paget’s disease of the breast?

Most surgeries for Paget’s disease of the breast result in disfigured nipples or breasts. Your doctor will talk to you about options for breast reconstruction surgery to restore these areas.

Prevention

What are the risk factors for Paget’s disease of the breast?

The risk factors for Paget’s disease of the breast are the same as for other types of breast cancer. People at higher risk for developing Paget’s disease include those who:

  • Are over the age of 55
  • Have a personal or family history of breast cancer or abnormalities
  • Inherited a gene defect associated with breast cancer
  • Have denser than-normal breast tissue on mammograms
  • Were exposed to radiation to the chest to treat other types of cancer
  • Are overweight
  • Take hormones for birth control or to relieve the symptoms of menopause
  • Drink excessive amounts of alcohol

Can Paget’s disease of the breast be prevented?

Steps you can take to reduce your risk of Paget’s disease and its complications include:

  • Exercise regularly.
  • Eat healthy foods.
  • Maintain a healthy weight.
  • Limit alcohol consumption.
  • Have regular breast checks and mammograms for early detection to prevent the spread of the disease.

Outlook / Prognosis

What is the prognosis (outlook) for people with Paget’s disease of the breast?

The outlook for people with Paget’s disease of the breast depends on where cancer is located. For people with underlying cancer in the breast, survival rates decline as the stage (how far it has spread) of that cancer increases. If cancer has not spread to surrounding tissue, the outlook is excellent.

Living With

When should I call the doctor if I suspect I have Paget's disease of the breast?

Contact your doctor if the skin on your nipple is itchy or irritated for more than a month.

What questions should I ask my doctor about Paget's disease of the breast?

If you have Paget’s disease of the breast, you may want to ask your doctor: These first two questions can usually only be answered after surgery. Most patients with Paget’s disease do not have distant spread of disease and staging is dependent on the findings at operation.

  • Have I been evaluated for cancer anywhere else in the breast or elsewhere in my body?
  • What stage is the cancer?
  • What type of surgery is best for me?
  • Will I need additional treatment after surgery?
  • What resources are available to support me?

When can I go back to my regular activities when I have been treated for Paget's disease of the breast?

Healing times for Paget’s disease of the breast depend upon the treatment. Your doctor will tell you when you can go back to daily activities.

Last reviewed by a Cleveland Clinic medical professional on 04/16/2019.

References

  • American Cancer Society. Accessed 4/17/2019.Paget Disease of the Nipple. (https://www.cancer.org/cancer/breast-cancer/understanding-a-breast-cancer-diagnosis/types-of-breast-cancer/paget-disease-of-the-nipple.html)
  • Breastcancer.org. Accessed 4/17/2019.Paget’s Disease of the Nipple. (https://www.breastcancer.org/symptoms/types/pagets)
  • National Cancer Institute. Accessed 4/17/2019.Paget Disease of the Breast. (https://www.cancer.gov/types/breast/paget-breast-fact-sheet)
  • National Institutes of Health. Accessed 4/17/2019.Paget’s Breast Disease: A Case Report and Review of the Literature. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5660109/)
  • The Susan G. Komen Breast Cancer Foundation Inc. Accessed 4/17/2019.Paget Disease of the Breast. (https://ww5.komen.org/BreastCancer/Pagets-Disease-of-the-Breast.html)

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