Key Points about Paget’s Disease of the Nipple

  • Paget’s disease of the nipple is a rare breast cancer that begins in the nipple and spreads to the areola.
  • Paget’s disease of the nipple usually affects the ducts of the nipple first.
  • Treatment for Paget’s disease of the nipple typically includes surgery, followed by other therapies such as chemotherapy, radiation therapy or hormone therapy.

Overview

Paget’s disease of the nipple is a rare type of breast cancer that begins on the nipple and then spreads to the areola (dark area surrounding the nipple). This condition is also known as Paget’s disease of the breast.

Paget’s disease of the nipple causes

The majority of people with Paget’s disease of the nipple also have ductal carcinoma in situ (DCIS) or invasive cancer in the breast. The cells from the ductal cancer break off from the original tumor and travel through the milk ducts to the nipple and areola.

Paget’s disease of the nipple risk factors

The following factors may increase your risk for developing Paget’s disease of the nipple:

  • Being obese
  • Being older than 50
  • Being previously exposed to radiation therapy
  • Drinking alcohol
  • Having a family history of breast cancer
  • Having ductal breast cancer – either invasive or noninvasive
  • Having lobular carcinoma in situ
  • Having never been pregnant
  • Having started your menstrual period before age 12
  • Having the BRCA1 or BRCA2 genes
  • Having your first child after age 30

Paget’s disease of the nipple symptoms

Signs and symptoms of this condition may include:

  • Crusty, hardened or oozing skin on the nipple and/or areola
  • Flaky or scaly skin on the nipple
  • Inverted (turned in) nipple
  • Itching
  • Lump in your breast
  • Nipple discharge that is straw-colored or bloody
  • Redness
  • Thickening of the skin on the breast
  • Tingling or burning feeling

In most cases, the above symptoms occur in only one breast.

Paget’s disease of the nipple diagnosis

Your doctor will use one or more of the following tests to diagnose Paget’s disease of the nipple:

  • Breast exam - your doctor will begin by performing a breast exam, in which he or she uses the pads of the fingers to carefully check your breasts for abnormalities.
  • Biopsy - during this test, your doctor removes a biopsy (tissue sample) from the suspicious area. The biopsy is sent to the lab for close analysis, where technicians can determine if there are any abnormalities present.
  • Breast magnetic resonance imaging (MRI) - an MRI uses magnets and radio waves to generate detailed images of the inside of the body. Before undergoing the breast MRI, you will receive an injection of a contrast dye to your vein. Contrast dye helps the images show up brighter and more clearly.
  • Breast ultrasound - this type of imaging test uses sound waves to create detailed images of the inside of your body. Your doctor can use ultrasound images to determine if there are any abnormalities present.
  • Mammogram - this type of imaging test is a specialized X-ray used to screen the breasts for abnormalities.

Paget’s disease of the nipple treatment

Depending on your personal health history, the extent of your Paget’s disease of the nipple and other factors, your oncologist may recommend one or more of the following treatment options:

  • Surgery - if you are diagnosed with Paget’s disease of the nipple, you will likely need to undergo surgery. Your surgeon will advise you on the type of surgery you will need to undergo, which will typically depend on the extent of your condition. Your surgeon will work to preserve as much surrounding healthy tissue as possible. In some cases, you may opt to undergo reconstructive surgery following surgery to treat breast cancer.
  • Chemotherapy - you may need to undergo chemotherapy after your surgical treatment. During this treatment, medication is used to destroy cancerous cells. Chemotherapy can be taken via an oral (by mouth) pill or intravenously (through a vein). Chemotherapy can help destroy any cancerous cells that your surgeon was not able to remove surgically.
  • Hormone therapy - your doctor may prescribe hormone medications, which can reduce your chance of recurrence of Paget’s disease of the nipple after you have undergone surgery to remove the affected area.
  • Radiation therapy - you may need to undergo radiation therapy after your surgery for Paget’s disease of the nipple. During this treatment, radiation is used to destroy any microscopic cancerous cells that could not be removed surgically.

When should I seek care?

If you experience any of these symptoms, start by voicing your concerns and symptoms to your primary care provider. From there, your doctor may suggest seeing an oncologist for more specialized treatment.

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