What is Paget’s Disease of the Breast?

Paget’s disease of the breast is a rare type of breast cancer found in the skin of the nipple or on the areola (dark circle of skin around the nipple). This type of cancer can be isolated to the nipple, but more commonly, will have one or more tumors in the same breast. The tumors are either ductal carcinoma in situ, or less commonly, it may be accompanied by invasive breast cancer in the milk ducts of the same breast (ductal carcinoma).

Paget’s disease of the breast typically occurs in women over 50. Many women who are diagnosed with Paget’s disease of the breast will have a lump behind the nipple and the majority of those cases are invasive breast cancer. The disease can affect men, but it is extremely rare. The primary form of treatment for those diagnosed with Paget’s disease of the breast is surgery.

Sometimes Paget’s disease of the breast is mistaken for common skin conditions, such as a rash or eczema. It may appear as a red scaly rash on the skin of the nipple and areola. The rash may be itchy and flakey. It may also cause discharge from the nipple. If the affected area is left untreated or it is scratched, it can bleed, scab over, or become ulcerated.

Signs and Symptoms

Typically, symptoms of Paget’s disease of the breast only affect one breast, but on rare occasions, they may affect both. Initially, symptoms may fluctuate but worsen over time. Symptoms may include:

• Nipple itching
• Nipple burning
• Dry, flaking or scaling skin of the nipple or areola
• Skin rash that may be raised, red, crusting, oozing (resembles weeping eczema), raw, or bleeding
• Bloody or pus-like nipple discharge
• Inflammation and soreness
• Flat or inverted nipple
• Noticeable breast lump under the skin

Causes

There is no known cause for Paget’s disease of the breast, however, genetics may play a role in increasing the risk of developing the disease. It has been theorized that Paget’s disease of the breast develops when the cancer cells of an underlying tumor in the breast travel through the milk ducts and into the nipple and areola. This theory would explain why people with Paget’s disease of the breast also have underlying tumors in the same breast.

Paget’s disease of the breast sometimes develops independently of other tumors in the breast. Additionally, tumors in the breast and Paget’s disease of the breast can sometimes develop independently of each other.

Risk Factors

Women over the age of 50 are more likely to develop Paget’s disease of the breast. Although extremely rare, it can also affect some men. Specific risk factors for Paget’s disease of the breast are the same as all breast cancer type risk factors. These include:

• Increasing age
• Personal history of breast conditions
• Personal history of breast cancer
• Inherited genes (BRCA1 and BRCA2 gene mutations)
• Family history of breast cancer
• Radiation exposure
• Obesity
• Drinking alcohol
• Beginning your menstrual cycle at a younger age (before age 12)
• Beginning menopause at an older age
• Having your first child at an older age (after 30)
• Having never been pregnant
• Receiving postmenopausal hormone therapy

Diagnosis

Paget’s disease of the breast is typically diagnosed by doing a nipple biopsy. There are several different types of nipple biopsies (surface, shave, punch, and wedge) and your doctor will consult with you to determine which procedure is best. Less commonly, a doctor may remove the entire nipple. Once the sample is examined, if cancer is confirmed, your doctor will check for any lumps or tumors in the breast itself. Your doctor will perform a breast exam and may likely recommend additional testing such as a mammogram, ultrasound, or MRI to determine if there are any underlying tumors in the breast.

Treatment

The main type of treatment for Paget’s disease of the nipple is surgery. Depending on if the cancer has spread and the size of the tumor(s), surgery may involve removing the entire breast (mastectomy) or surgery may only remove the nipple and areola and any affected tissue underneath them (central excision). A lumpectomy, also known as breast-conserving surgery, removes the cancer and a small area of healthy tissue around it. If the whole breast is removed, your doctor will discuss breast reconstruction surgery with you.

Additionally, your doctor may recommend removing a limited number of lymph nodes (sentinel node biopsy) or removing several lymph nodes (axillary lymph node dissection) if the cancer is suspected to have spread to your lymph nodes.

If the cancer is more invasive, surgery may be combined with other treatments. Other treatments include:

• Chemotherapy. This treatment uses strong medications to destroy cancer cells.
• Radiotherapy. This treatment administers controlled doses of radiation to destroy cancer cells.
• Targeted or hormone therapy. These treatments reduce the risk of the cancer returning and reduce the risk of the cancer spreading.

If Paget’s disease of the breast is caught in its early stages, the prognosis for a full recovery is more likely.

Prevention

There is no definite way to prevent cancer, but there are certain preventative measures that may reduce the risk of developing cancer or may allow you to detect it in its earlier stages. Prevention measures include:

• Maintaining a healthy weight
• Regular exercise
• Limit your intake of alcohol
• Breastfeeding may lower your risk of breast cancer
• Knowing your family medical history
• Screening for cancer gene mutations
• Routine breast exams and mammograms
• Medications that block estrogen
• Breast removal surgery (prophylactic mastectomy)

Complications

Breast cancer treatments can have lingering side effects. Some side effects may last days, weeks, or years after treatment is finished. Complications include:

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