Paget's disease of the breast

Definition

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 in women older than age 50. Most women with Paget's disease of the breast have underlying ductal breast cancer (in situ or, less commonly, invasive). Only in rare cases is the cancer confined to the nipple itself.

Nipple changes

Nipple changes

Breast and nipple changes can be a sign of breast cancer. Make an appointment with your doctor if you notice anything unusual. ...

Symptoms

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:

• Flaky or scaly skin on your nipple
• Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
• Itching
• Redness
• A tingling or burning sensation
• Straw-colored or bloody nipple discharge
• A flattened or turned-in nipple
• A lump in the breast
• Thickening skin on the breast

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. In rare cases, only the areola is affected.

The skin changes may fluctuate early on, making it appear as if your skin is healing on its own. On average, women experience signs and symptoms for six to eight months before a diagnosis is made.

When to see a doctor
Check your nipple and areola on both breasts on a regular basis, such as during breast self-exams. If you feel a lump, or if you experience itching or skin irritation that persists for more than a month, see 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. You may need a biopsy — a procedure that collects a small tissue sample for microscopic analysis — to evaluate the affected area.

Nipple changes

Nipple changes

Breast and nipple changes can be a sign of breast cancer. Make an appointment with your doctor if you notice anything unusual. ...

Causes

Doctors don't know what causes Paget's disease of the breast. But the most widely accepted theory is that the disease results from an underlying ductal breast cancer. Cancer cells from the original tumor travel through milk ducts to the nipple and its surrounding skin.

Risk factors

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:

• Age. Your chances of developing breast cancer increase as you get older.
• A personal history of breast cancer. If you've had breast cancer in one breast, you have an increased risk of developing cancer in the other breast.
• A personal history of breast abnormalities. If you've had lobular carcinoma in situ or atypical hyperplasia, your risk of developing breast cancer is higher. Certain benign breast conditions also are associated with a slightly increased risk.
• Family history. If you have a mother, sister or daughter with breast or ovarian cancer or both, or even a father or brother with breast cancer, you have a greater chance of developing breast cancer.
• Genetic predisposition. Defects in one of several genes, especially BRCA1 or BRCA2, put you at greater risk of developing breast cancer as well as ovarian and other cancers.
• Dense breast tissue. Women with dense breast tissue, as seen on a mammogram, face a higher risk of breast cancer.
• Radiation exposure. If you received radiation treatments to your chest as a child or young adult to treat another cancer, you're more likely to develop breast cancer later in life.
• Excess weight. Weighing more than is healthy for your age and height increases your risk of breast cancer — especially after menopause and if you gained weight as an adult.
• Hormone replacement. Taking estrogen after menopause increases the risk of breast cancer for some women.
• Race. White women are more likely to develop breast cancer than black or Hispanic women are, but black women are more likely to die of the disease.

Having one or more risk factors doesn't necessarily mean you'll develop breast cancer. Most women with breast cancer have no known risk factors.

Preparing for your appointment

If you suspect you have Paget's disease of the breast, your initial appointment might be with your family doctor. Or, when you call to set up an appointment, you may be referred directly to a doctor who specializes in treating breast conditions.

What you can do
To prepare for your appointment:

• Take note of all your symptoms, even if they seem unrelated to your breast.
• Make a list of all medications, vitamins and supplements that you regularly take.
• Write down questions to ask your doctor. List your questions from most important to least important in case time runs out.

What to expect from your doctor
Your doctor may ask you questions about:

• The nature of the skin changes on your nipple
• Whether you also experience nipple discharge, bleeding, burning or itching
• If you have any other breast signs and symptoms, such as a breast lump or area of thickening
• If you have any breast pain
• How long you've experienced signs and symptoms

Your doctor may also ask questions about your personal and family medical history and other possible risk factors for breast cancer.

Tests and diagnosis

Tests to evaluate your condition may include:

• Clinical breast exam and physical exam. During this exam, your doctor physically checks for unusual areas in both breasts, noting the appearance of the skin on and around your nipples and feeling for any lumps or areas of thickening.
• Mammography. Mammography — an X-ray exam of your breast tissue — may indicate whether the nipple and skin changes are linked to an underlying breast cancer, as is usually the case in Paget's disease of the breast. If results from mammography in both breasts don't reveal any signs of breast cancer, your doctor may follow up with magnetic resonance imaging (MRI), which may detect cancer that's not able to be seen on a mammogram.
• Breast biopsy. During a biopsy, your doctor obtains a small sample of tissue from the skin of your nipple for examination under a microscope. If you have nipple discharge, a sample of the discharge might also be collected. If you have a breast lump, a biopsy of that tissue will also be done. If cancer cells are detected in the samples collected, you may be referred to a breast surgeon to discuss treatment options.
• Sentinel lymph node biopsy. If you have invasive breast cancer, the lymph nodes under your arm (axillary lymph nodes) need to be checked to see if the cancer has spread to this area. This can be done in a procedure known as a sentinel lymph node biopsy. During the biopsy, your surgeon locates the sentinel nodes — the first lymph nodes to receive the drainage from breast tumors and therefore the first place cancer cells will travel. If a sentinel node is removed, examined and found to be normal, the chance of finding cancer in any of the remaining nodes is small and no other nodes need to be removed.

Receiving a mammogram

Receiving a mammogram

During a mammogram, you stand in front of an X-ray machine designed for mammography. A technician places your breast on a platform that holds the X-ray film and positions the platform to match your ...

Ultrasound-guided breast biopsy

Ultrasound-guided breast biopsy

During an ultrasound-guided breast biopsy, your radiologist places an ultrasound probe on your breast to locate the breast abnormality. Then, he or she inserts a thin, hollow needle to obtain tissue ...

Sentinel node biopsy

Sentinel node biopsy

To determine whether cancer has spread to the nearby lymph nodes, doctors may use a sentinel node biopsy. Doctors determine which lymph node is likely to be the first stop for spreading cancer ...

Treatments and drugs

Paget's disease of the breast usually requires surgery. Which type of surgery you need depends on the condition of the skin around your nipple and how advanced the underlying cancer is.

Surgical options include:

• Simple mastectomy. This procedure involves removing your entire breast, but not the lymph nodes in your armpit (axillary lymph nodes). Simple mastectomy might be recommended in cases in which an underlying breast cancer exists but hasn't spread to the lymph nodes.
• Lumpectomy. Breast-conserving surgery (lumpectomy) involves removing only the diseased portion of your breast. Your surgeon removes your nipple and areola along with a wedge- or cone-shaped section of your breast. The surgeon focuses on removing as little breast tissue as possible, while ensuring that the tissue removed includes an outer margin free of cancer cells so that only healthy cells remain. Lumpectomy to treat Paget's disease of the breast requires follow-up radiation therapy. Lumpectomy wouldn't be recommended if you can't have radiation therapy for some reason. Most women have nipple reconstruction after their treatment.

Adjuvant therapy
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 tumor tests positive for certain characteristics, such as having estrogen or progesterone receptors.

Simple mastectomy and modified radical mastectomy

Simple mastectomy and modified radical mastectomy

A simple, or total, mastectomy (left) removes the breast tissue, nipple, areola and skin, but not all the lymph nodes. A modified radical mastectomy (right) removes the entire breast, including ...

Lumpectomy

Lumpectomy

During a lumpectomy, your surgeon makes an incision large enough to remove the tumor and a margin of healthy tissue surrounding the tumor. The rest of your breast remains intact. ...

Coping and support

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.

Every woman finds her own way of coping with a breast 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 the details of your cancer — 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 women 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 women 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 the Breast Cancer Network of Strength and CancerCare.
• Find someone to talk about your feelings with. Find a friend or family member who is a good listener or talk with a clergy member or counselor. Ask your doctor for a referral to a counselor or other professional who works with cancer survivors.
• 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.
• Maintain intimacy with your partner. In Western cultures, women's breasts are associated with attractiveness, femininity and sexuality. Because of these attitudes, breast cancer may affect your self-image and erode your confidence in intimate relationships. Talk to your partner about your insecurities and your feelings.
• Take care of yourself. Make your well-being a priority during cancer treatment. Get enough sleep so that you wake feeling rested, choose a diet full of fruits and vegetables, make time for gentle exercise on days you feel up to it and find time for things you enjoy, such as reading or listening to music. If you need to, be prepared to relinquish your role as caretaker for a while. This doesn't mean you're helpless or weak. It means you're using all your energy to get well.

Prevention

You can't avoid certain risk factors for breast cancer, such as your age, genetics or family history. But other factors that could increase your chances of developing breast cancer are within your control. Healthy lifestyle habits, such as getting enough exercise, limiting alcohol and eating a healthy diet, may offer you some protection from breast cancer.

Certain medications and surgical procedures may help reduce your chance of developing breast cancer. Preventive medications do have potential side effects, however, and risk-reducing surgery isn't the best option for everyone. Discuss your personal risk of developing breast cancer with your doctor and ask whether you would be a candidate for therapy that may reduce that risk.