An Overview of Paget's Disease of the Breast

Paget’s disease of the breast (also known as Paget’s disease of the nipple) is a rare type of breast cancer in which cancer cells are found on the surface of the nipple and the dark circle surrounding it (the areola). Paget’s disease of the breast itself does not cause breast lumps or tumors. However, the majority of people with Paget's have either invasive breast cancer or ductal carcinoma in situ (DCIS), and related breast tumors are almost always found in the same breast that has Paget's disease cells. 

Paget’s accounts for 5% of all breast cancers. It most commonly affects people in their 50s, especially menopausal women. That said, the disease may also appear in adolescents and the elderly. Male breast cancers only account for 0.5% of breast cancers, and Paget’s in men is even rarer.  

Paget’s disease causes the skin on the nipple and the area around it to be red, sore, flaky, and scaly. Early on, these symptoms will come and go.

Symptoms will worsen with time and may include:

• Itching or a burning sensation in the nipple and/or areola
• Pain and sensitivity
• Flattening of the nipple
• Yellow or bloody discharge from the nipple
• A lump in the affected area

If you have any of the signs or symptoms noted above, see you doctor as soon as possible.

Doctors don't know exactly what causes Paget’s disease of the breast. Most researchers think the disease is the result of an underlying breast issue; more than 97% of people with Paget's also have DCIS or invasive cancer somewhere else in the breast, according to the nonprofit Breastcancer.org.

Cancer cells may travel from the tumor up through the milk ducts, where they enter the nipple and areola.

In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. In these cases, nipple skin cells may spontaneously change into cancer cells.

Risk factors that increase the risk of any type of breast cancer are also associated with Paget’s disease. These include:

• Increasing age
• Race: White women have a higher risk of breast cancer than black and Hispanic women.
• Family history (mother, sister, or daughter) of breast or ovarian cancer
• Personal history of breast cancer in one breast, lobular carcinoma in situ (LCIS), atypical hyperplasia, or certain benign breast conditions
• Inherited gene mutation (e.g., BRCA1 or BRCA2)
• Dense breast tissue
• Previous radiation exposure to the chest
• Use of hormone replacement therapy after menopause
• Being overweight, especially after menopause
• Excessive alcohol consumption

If you are experiencing any of the above signs and symptoms, see a health professional. Diagnosing Paget's disease will involve the following steps: 

• Clinical breast exam: Up to 50% of people with Paget's disease of the nipple have a breast lump or an area of thickening that is felt upon clinical exam. 
• Mammography: In Paget’s disease of the breast, a mammogram may reveal nipple and skin changes that are linked to underlying breast cancer. If a mammography of both breasts doesn’t show anything unusual, your doctor may follow up with magnetic resonance imaging (MRI).
• Breast biopsy: During a breast biopsy, a small sample of tissue is taken from the skin of the nipple for examination under a microscope (a sample will also be taken from the lump, if present). If there is nipple discharge, it will be collected and examined as well.
• Sentinel lymph node biopsy: In this procedure, a surgeon locates and removes sentinel nodes—the first place breast cancer cells go. If a sentinel node is examined and found to be negative, it is unlikely that cancer will be found in the remaining nodes.

If cancer cells are detected in any of the biopsy samples, your doctor will refer you to a surgeon to discuss treatment.

Surgery is the primary treatment option for Paget's disease of the breast. There are two options:

• Lumpectomy: This breast-conserving surgery removes only the diseased portion of the breast. With Paget’s disease, the surgeon removes the nipple and areola, along with a cone-shape part of the breast. Care is taken to spare as much tissue as possible while ensuring that the cancer cells are removed.
• Mastectomy: If the underlying breast cancer is invasive and removing the entire breast makes more sense, a mastectomy will be done instead. If lymph nodes are affected, these will be removed with a sentinel lymph node biopsy.

After surgery, your doctor may recommend additional treatment with anti-cancer drugs (chemotherapy), radiation, or hormone therapy to prevent a breast cancer recurrence. 

For example, if a lumpectomy surgery is used to treat Paget’s disease of the breast, it is usually followed with radiation therapy to kill any cancer cells that may be in the remaining breast tissue. Many women will have nipple reconstruction after their lumpectomy and once radiation is completed. 

The specific treatment that's recommended will depend on the extent of the cancer and the nature of the tumor (for example, if the tumor has estrogen or progesterone receptor).

Since Paget's disease is not common, you've likely heard less about it than more common types of breast cancer. In addition, it can be difficult to find a Paget's support group in many communities due to the limited number of people with the disease. Fortunately, online communities give you the ability to connect with people around the world who share your diagnosis. Consider reaching out for support from others facing your same challenges.