Paget's Disease of the Breast

What is Paget's disease of the breast?

Paget's disease of the breast is a rare type of cancer that develops in the skin of your nipple, and sometimes the areola (the darker skin surrounding the nipple). It may be isolated to the nipple as stage 0 breast cancer (ductal carcinoma in situ), or it may be accompanied by invasive breast cancer in the milk ducts of the same breast. It’s also called mammary Paget's disease.

Paget's disease of the breast resembles eczema on your nipple and may be mistaken for an ordinary rash at first. It may cause itching and scaling, red or raised plaques on your skin and discharge from the nipple. Most people who have these symptoms don’t have Paget's disease. However, if you do, these may be the first noticeable signs of underlying breast cancer.

Who gets Paget's disease of the breast?

Paget's disease of the breast almost exclusively affects individuals who were assigned female at birth, although rare cases have been reported in people assigned male at birth. The average age of diagnosis is 57, but it has been found in people much younger and older, too. Less than 4% of breast cancer cases involve Paget's disease of the breast.

How quickly or aggressively does Paget's disease of the breast progress?

That depends on whether or not you have underlying ductal carcinoma, and what stage it’s in. If you only have cancer in your milk ducts and it hasn’t spread to the surrounding breast tissues, it’s called “ductal carcinoma in situ” (DCIS). This is considered stage 0. This type of cancer is treated with surgical removal of the tumor(s) and nipple, followed by radiation.

When ductal carcinoma does spread beyond the milk ducts, it’s called infiltrating or invasive ductal carcinoma. This cancer progresses in stages. In the early stages, you can still treat it by removing the affected breast tissue and axillary lymph nodes. Invasive breast cancer becomes more aggressive and difficult to treat in the later stages when it spreads beyond the breast.

What are the symptoms of Paget's disease of the breast?

Symptoms affect your nipple and/or areola. They usually only affect one breast, but rarely, they may affect both. They may appear to come and go at first but eventually worsen. Symptoms can include:

• Nipple itching that can turn to burning.
• Dry skin, flaking or scaling of the nipple or areola.
• Skin rash that might be red, raised, crusting, raw or bleeding.
• Inflammation and soreness.
• Crusting and oozing resembling weeping eczema.
• Bloody or pus-like nipple discharge.
• Flat or inverted nipple.
• Palpable breast lump under the skin.

What causes Paget's disease of the breast?

What causes cancer, in general, is still a mystery to scientists, but ongoing research continues to turn up new clues. We know that it involves abnormal changes to certain cells in your body that then divide and replicate themselves in an uncontrolled manner. Current research suggests that these cellular changes may begin in your DNA or genetic code. But we don’t know why these changes occur when they do.

Scientists have noticed that certain “risk factors” are often present when cancer occurs, and they believe these factors might contribute in some way. For example, you’re more likely to get cancer if you’ve been exposed to certain environmental toxins, or if you smoke or drink alcohol more frequently. However, 85% of breast cancer occurs in people with no known risk factors.

How is Paget's disease of the breast diagnosed?

Your healthcare provider may attempt to treat your condition as eczema at first. When that fails, or when they suspect Paget's disease of the breast, they’ll need to take a sample of the nipple tissue to examine under a microscope. Paget's cells are easily recognizable this way. They’ll likely take a punch biopsy, which means using a circular cutting tool like a hole punch to remove a small, disk-shaped piece.

After identifying Paget's disease of the breast, your healthcare provider will need to find out if you have underlying ductal carcinoma, and if so, how advanced it is. They’ll start by examining your breast tissue with imaging tests, such as a mammogram or breast ultrasound. If they find an area suspicious for underlying cancer, they will recommend a needle biopsy to take a sample of the breast tissue.

How is Paget's disease of the breast treated?

Treatment involves surgery and sometimes additional cancer therapies.

Breast cancer surgery

Any cancer in your breast will need to be surgically removed, but how much tissue is removed will depend on your condition. If you only have Paget's disease of the breast, and no other tumors, you may have more minimal, breast-conserving surgery. That means your surgeon will aim to remove only the involved tissue. They’ll remove your nipple and areola with a small margin of normal tissue around it.

Depending on how extensive the cancer is, your surgeon may recommend a mastectomy as well. You may have breast reconstruction surgery afterward if you choose.

Adjuvant therapy

After surgery, you’ll likely have additional, complementary treatments to prevent cancer from recurring. Adjuvant therapies may include:

• Radiation therapy.
• Chemotherapy.
• Hormone therapy.
• Immunotherapy.

Can Paget's disease of the breast be cured?

Breast cancer survival is based on the stage of the cancer when it’s detected. If you only have Paget's disease of the nipple or ductal carcinoma in situ (stage 0) that hasn’t spread beyond the milk ducts, your prognosis is excellent. Most people who discover and treat these conditions early will make a full recovery. Once breast cancer begins to spread, it becomes progressively harder to beat.

When should I see my healthcare provider about Paget's disease of the breast?

Always see your healthcare provider if you notice any changes in your breast, including skin conditions and changes under the skin. Most of the time, it won’t be cancer, but it’s always best to check. If you have eczema-like symptoms on your nipple or areola and you’ve tried to treat them but the treatments aren’t helping, make an appointment with your healthcare provider for follow-up testing.

How should I take care of myself if I have this disease?

The best way to take care of yourself is to begin treatment as soon as possible. But you’ll also need support. Try to enlist at least one support person to talk to, to accompany you to your appointments and/or to help you manage at home. Write a list of questions to bring to your doctor and come prepared to take notes, or let your support person take them. Then you can review them together afterward.

Is Paget's disease of the breast related to other Paget's diseases?

An English surgeon named Sir James Paget made a number of medical discoveries in the late 19th century. There are a few diseases named for him, but they’re not all related. For example, Paget's disease of the bone is a non-cancerous bone disease, unrelated to Paget's disease of the breast. However, another rare cancer called extramammary Paget's disease (EMPD) is similar in nature.

Paget's disease of the breast is characterized by distinctive cancer cells on the skin of the nipple that can be seen under a microscope. Because Paget originally described them, they’re known as Paget cells. Extramammary Paget's disease is also characterized by Paget cells. It produces a similar rash, but on the genitals instead of the nipple. The two diseases are similar but separate. They don’t occur together.

A note from Cleveland Clinic

Paget's disease of the breast is early-stage breast cancer of the milk ducts that occurs in the nipple. It’s sometimes accompanied by more invasive breast cancer. You may notice nipple changes such as crusting, flaking of the skin, discharge or a raw area that doesn’t heal. The earlier you identify and treat it, the better your outcome will be. Many people make a full recovery, but it will require surgery to remove it.