Paget’s Disease of the Breast

Paget’s disease of the breast is an uncommon type of cancer that affects the nipple and surrounding skin (areola). Because the condition is so rare, it is important to seek specialized treatment to ensure the best possible outcome.

Tampa General Hospital’s Cancer Institute is home to one of the highest-volume breast cancer programs in the state of Florida. The board-certified surgeons in our Breast Cancer Center are skilled and experienced in performing the latest minimally invasive procedures to eliminate all traces of breast cancer while preserving as much breast tissue as possible.

What Causes Paget’s Disease of the Breast?

Scientists do not yet fully understand what causes Paget's disease of the breast. Although the nipple or areola may become cancerous on their own, most people with this diagnosis also have ductal carcinoma in situ or invasive breast cancer in the same breast. For this reason, many experts believe Paget’s disease develops when cancerous cells break away from a tumor inside the breast and travel through the milk ducts to reach the nipple and areola.

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

The early signs of Paget’s disease of the breast are often similar to those of a benign skin condition, such as eczema or dermatitis, and can include:

  • Redness, itching or tingling sensations around a nipple or areola
  • Flaky, crusty or thickened skin on or around a nipple
  • A flattened or inverted nipple
  • Yellowish or bloody discharge from a nipple

Initially, the breast skin changes may come and go and improve with topical treatment, making it seem as if the irritation is healing. For this reason, it is especially important to discuss persistent symptoms with a physician.

How Is Paget’s Disease of the Breast Diagnosed?

If Paget’s disease of the breast is suspected based on the symptoms, a physician will typically order a nipple biopsy. Some options include a:

  • Surface biopsy – A physician uses a glass slide or another tool to gently scrape cells away from the surface of the skin.
  • Shave biopsy – A physician uses a razor-like tool to remove the top layer of skin.
  • Punch biopsy – A physician uses a circular cutting tool (punch) to remove a disk-shaped piece of tissue.
  • Wedge biopsy – A physician uses a scalpel to remove a small wedge of tissue.

The tissue sample will then be sent to a lab for a microscopic evaluation by a pathologist.

In addition to ordering a nipple biopsy, the physician will perform a clinical breast examination to check for lumps or other unusual changes. Based on the result, the physician may order additional diagnostic testing, such as a mammogram, ultrasound or magnetic resonance imaging (MRI) scan.

How Is Paget’s Disease of the Breast Treated?

In years past, a mastectomy with or without an axillary lymph node dissection was the first-line treatment for Paget’s disease of the breast. The reason was that most patients had at least one tumor inside the same breast that was located far enough away from the nipple and areola that it could not be removed by surgery performed on the nipple and areola alone.

However, recent studies show that a combination of breast-conserving surgery, including removal of the nipple and areola, and whole-breast radiation therapy can be an effective option for patients with Paget’s disease of the breast who do not have a breast lump that can be felt or seen in a mammogram. And for those who have a breast tumor and undergo a mastectomy, a sentinel lymph node biopsy may be performed to check whether cancerous cells have spread to the axillary lymph nodes before a more extensive axillary lymph node dissection is considered.

For enhanced treatment effectiveness, surgery may be followed by chemotherapy and/or hormone therapy depending on the stage of the breast tumor and other factors, such as the presence or absence of:

  • Lymph node involvement
  • Estrogen and/or progesterone receptors in the tumor cells
  • HER2 protein overexpression in the tumor cells

Benefit From World-Class Care at TGH

The experienced and compassionate specialists at TGH can help you understand the implications of your breast cancer diagnosis and provide in-depth information about your treatment options for Paget’s disease of the breast, including breast-conserving surgery. If would like to talk with an expert on our team, contact us at (800) 844-4554 to request an appointment.