Paget's disease of the breast

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 3, 2016
StartPaget's disease of the breast

Paget's disease of the breast is a form of breast cancer that affects the skin of the patient's nipple. Paget's disease of the breast will usually impact the darker patch of skin (the areola) around the nipple of a person's breast.

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Definition & Facts

Generally, individuals who have this type of cancer will also house one or several tumors within the same breast impacted by the condition. These tumors are either invasive breast cancer or ductal carcinoma in situ.

One to four percent of women who have breast cancer also have Paget's disease of the breast. The five-year survival rate of women who have Paget's disease of the breast but do not have invasive cancer is over 80 percent.

Symptoms & Complaints

Individuals who are attempting to determine whether they have Paget's disease of the breast should know that the condition is marked by several symptoms. Some of them include:

  • Thickened, crusty, or flaking skin around or on the nipple
  • Redness, tingling, or itching in the areola and/or nipple 
  • Discharge (oftentimes bloody or yellowish) from the nipple 
  • A flattened nipple

In many cases, people with Paget's disease of the breast experience symptoms for many months before they are diagnosed correctly. 

Causes

The causes of Paget's disease of the breast are not fully known. However, one of the more prevalent theories holds that the cancerous cells from the tumor within the breast travel into the patient's milk ducts and then through the nipple and areola. This theory offers an explanation for why tumors and Paget's disease of the breast almost always occur together.

Another theory regarding the disease holds that cells within the areola or nipple become cancerous all on their own. This explanation would account for why some people acquire Paget's disease of the breast yet do not have a tumor within the affected breast. Also, it might be possible for tumors and Paget's disease of the breast to develop independently within the same breast.

Diagnosis & Tests

Paget's disease of the breast is diagnosed through a biopsy. There are several kinds of biopsies performed on the nipple, some of which include:

  • Shave biopsy: With this type of biopsy, a tool similar to a razor is used to remove the patient's top layer of skin. 
  • Surface biopsy: This form of skin biopsy involves using a glass slide to scrape cells off the skin's surface. 
  • Wedge biopsy: This procedure involves using a scalpel to remove a tiny wedge of the patient's tissue.
  • Punch biopsy: With this procedure, the doctor utilizes a circulating tool called a punch to remove a piece of tissue from the skin.

In some instances, the doctor might remove the patient's entire nipple to complete the biopsy. After this process is completed, the pathologist will examine the tissue or cells beneath the microscope to detect cancerous cells.

In most cases, individuals who have Paget's disease of the breast will also house one or several tumors within the same breast. Thus in addition to completing a nipple biopsy, the doctor will likely complete a breast examination.

The clinical breast exam will help the doctor detect any breast lumps or other significant changes. Nearly 50% of people with Paget's disease of the breast will also have a breast lump. There are several other diagnostic tests that the doctor might order, including magnetic resonance imaging (MRI), an ultrasound exam, or a diagnostic mammogram. These tests will help the doctor detect any tumors that may be present. 

Individuals who have Paget's disease of the breast as well as a breast tumor and plan to have a mastectomy should undergo a lymph node biopsy of the sentinel lymph nodes. This biopsy will help determine whether the cancer is contained or has metastasized. If cancerous cells are present in these lymph nodes, it is likely surgery is required.

Treatment & Therapy

In the past, the standard form of treatment for Paget's disease of the breast was mastectomy. The mastectomy is a form of surgery in which the patient's entire breast is removed. This will often include the areola and nipple. This is because individuals with Paget's disease of the breast generally have one or several tumors within the affected breast, and such tumors are often located many centimeters away from the areola and nipple.

This procedure could be accompanied with or without the removal of the lymph nodes (lymphadenectomy) on the same side of the chest impacted by the disease. This aspect of the procedure is referred to as axillary lymph node dissection.

While the aforementioned treatment modalities are common, studies point towards other alternatives. Specifically, a breast-conserving surgery that involves removing the areola and nipple while following up with radiation therapy as a form of adjuvant therapy for the whole breast is a safe option. However, this treatment path should only be pursued by patients whose mammograms reveal no tumor and who don't have a lump in the breast.

Depending on the features of the breast tumor as well as its stage, several other treatments may be recommended. Some of them include hormonal therapy and chemotherapy.

Prevention & Prophylaxis

There are several prevention strategies that can be implemented to prevent the onset of Paget's disease of the breast. Some of them include:

Another form of prophylaxis available to individuals attempting to avoid Paget's disease of the breast is the use of preventive medications. These estrogen-blocking medications help reduce the user's susceptibility to breast cancer. Some options include raloxifene and tamoxifen. It's important to note that these medications bring several side effects. As such, they are typically recommended for individuals who are at a high risk of developing breast cancer.

One final form of prophylaxis individuals can choose is preventive surgery. Women who are at a great risk of developing breast cancer may opt to undergo a preventive mastectomy. This surgery will remove their healthy breasts. Another option is the prophylactic oophorectomy, in which healthy ovaries are removed. This procedure will reduce susceptibility to both ovarian cancer and breast cancer.