This rare form of breast cancer causes breast skin changes. Learn about the symptoms, causes, diagnosis and treatments, including surgery.
Paget's (PAJ-its) disease of the breast is a rare form of breast cancer. Paget's disease of the breast starts on the nipple and extends to the dark circle of skin (areola) around the nipple. Paget's disease of the breast isn't related to Paget's disease of the bone, a metabolic bone disease.
Paget's disease of the breast occurs most often after age 50. Most people with this diagnosis also have underlying ductal breast cancer, either in situ — meaning in its original place — or, less commonly, invasive breast cancer. Only rarely is Paget's disease of the breast confined to the nipple itself.
Paget's disease of the breast affects your nipple and usually the skin (areola) surrounding it. It's easy to mistake the signs and symptoms of Paget's disease of the breast for skin irritation (dermatitis) or another noncancerous (benign) skin condition.
Possible signs and symptoms of Paget's disease of the breast include:
Signs and symptoms usually occur in one breast only. The disease typically starts in the nipple and may spread to the areola and other areas of the breast.
The skin changes may come and go early on or respond to topical treatment, making it appear as if your skin is healing. On average, signs and symptoms occur for several months before a diagnosis is made.
Be aware of any changes in your breasts. If you feel a lump in your breast, or if you experience itching or skin irritation that persists for more than a month, make an appointment with your doctor.
If you're being treated for a skin injury on your breast and the condition doesn't go away with treatment, make a follow-up appointment with your doctor.
Breast and nipple changes can be a sign of breast cancer. Make an appointment with your doctor if you notice anything unusual.
Doctors don't know what causes Paget's disease of the breast. The most widely accepted theory is that the disease results from an underlying ductal breast cancer. The cancer cells from the original tumor then travel through milk ducts to the nipple and its surrounding skin. Another theory is that the disease can develop independently in the nipple.
Risk factors that affect your likelihood of developing Paget's disease of the breast are the same factors that affect your risk of developing any other type of breast cancer.
Some factors that make you more susceptible to breast cancer include:
Making changes in your daily life may help reduce your risk of breast cancer. Try to:
Ask your doctor about breast cancer screening. Discuss with your doctor when to begin breast cancer screening exams and tests, such as clinical breast exams and mammograms.
Talk to your doctor about the benefits and risks of screening. Together, you can decide what breast cancer screening strategies are right for you.
Become familiar with your breasts through breast self-exam for breast awareness. You may choose to become familiar with your breasts by occasionally inspecting your breasts during a breast self-exam for breast awareness. If there is a new change, lumps or other unusual signs in your breast, talk to your doctor promptly.
Breast awareness can't prevent breast cancer, but it may help you to better understand the normal changes that your breasts undergo and identify any unusual signs and symptoms.
Limit postmenopausal hormone therapy. Combination hormone therapy may increase the risk of breast cancer. Talk with your doctor about the benefits and risks of hormone therapy.
You may experience bothersome signs and symptoms during menopause and, for you, the increased risk of breast cancer may be acceptable in order to relieve menopause signs and symptoms.
To reduce the risk of breast cancer, use the lowest dose of hormone therapy possible for the shortest amount of time.
If your doctor has determined that you have an increased risk of breast cancer, you may discuss options to reduce your risk, such as:
Preventive medications (chemoprevention). Estrogen-blocking medications reduce the risk of breast cancer if you have a high risk of the disease.
These medications carry a risk of side effects, so doctors reserve these medications for those who have a very high risk of breast cancer. Discuss the benefits and risks with your doctor.
Tests and procedures used to diagnose Paget's disease of the breast include:
Other tests and procedures may be used depending on your situation.
During a mammogram, you stand in front of an X-ray machine designed for mammography. A technician places your breast on a platform and positions the platform to match your height. The technician helps you position your head, arms and torso to allow an unobstructed view of your breast.
If you have Paget's disease of the breast, you will likely need surgery. The type of surgery depends on the condition of the skin around your nipple and how advanced the underlying cancer is.
Surgical options include:
Removing a limited number of lymph nodes (sentinel node biopsy). To determine whether cancer has spread to your lymph nodes, your surgeon will discuss with you the role of removing the lymph nodes that are the first to receive the lymph drainage from your cancer.
If no cancer is found in those lymph nodes, the chance of finding cancer in any of the remaining lymph nodes is small and no other nodes need to be removed.
You may choose to have breast reconstruction after surgery. Discuss your options and preferences with your surgeon.
Consider a referral to a plastic surgeon before your surgery. Your options may include reconstruction with a breast implant or reconstruction using your own tissue. These operations can be performed at the time of your mastectomy or at a later date.
After your operation, your doctor may recommend additional treatment (adjuvant therapy) with anti-cancer drugs (chemotherapy), radiation therapy or hormone therapy to prevent a recurrence of breast cancer.
Your specific treatment will depend on the extent of the cancer and whether your cancer tests positive for certain characteristics, such as having estrogen or progesterone receptors.
During a total (simple) mastectomy, the surgeon removes the breast tissue, nipple, areola and skin. Other mastectomy procedures may leave some parts of the breast, such as the skin or the nipple. Surgery to create a new breast is optional and can be done at the same time as your mastectomy surgery or it can be done later.
A breast cancer diagnosis can be overwhelming. And just when you're trying to cope with the shock and the fears about your future, you're asked to make important decisions about your treatment.
Everyone finds a personal way of coping with a cancer diagnosis. Until you find what works for you, it might help to:
Learn what you need to know about your breast cancer. If you'd like to know more about your breast cancer, ask your doctor for details — the type, stage and hormone receptor status. Ask for good sources of up-to-date information on your treatment options.
Knowing more about your cancer and your options may help you feel more confident when making treatment decisions. Still, some people may not want to know the details of their cancer. If this is how you feel, let your doctor know that, too.
Talk with other breast cancer survivors. You may find it helpful and encouraging to talk to other people with breast cancer. Contact the American Cancer Society to find out about support groups in your area.
Organizations that can connect you with other cancer survivors online or by phone include After Breast Cancer Diagnosis and CancerCare.
Keep your friends and family close. Your friends and family can provide a crucial support network for you during your cancer treatment.
As you begin telling people about your breast cancer diagnosis, you'll likely get many offers for help. Think ahead about things you may want help with, whether it's having someone to talk to if you're feeling low or getting help preparing meals.
If you suspect that you have Paget's disease of the breast, your initial appointment might be with your family doctor. Or, when you call for an appointment, you may be referred directly to a doctor who specializes in treating breast conditions.
To prepare for your appointment:
Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. Some basic questions to ask include:
Your doctor may ask you questions about:
Your doctor may also ask questions about your personal and family medical history and other possible risk factors for breast cancer.