Paget's disease of the breast
This information is about a condition called Paget’s disease of the breast. It should ideally be read with our general information about breast cancer and ductal carcinoma in situ (DCIS).
Paget's disease of the breast
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Paget’s disease of the breast is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have it (90%) have an underlying breast cancer. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in situ (DCIS). In DCIS, the cancer cells are completely contained within the milk ducts. In invasive breast cancer, the cancer starts in the ducts or lobes of the breast, and spreads into surrounding breast tissue.
Paget’s disease affects about 1-2 out of every 100 women with breast cancer (1-2%). It's most common in women in their 50s, but can occur at a younger or older age. It can affect men, but this is extremely rare.
Causes of Paget's disease
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The cause of Paget's disease is unknown, but certain women seem to be at a higher risk of developing breast cancer. This includes women who have never had children or had them late in life, women who started their periods at a young age or who had a late menopause, and women who have a strong family history of breast cancer.
Signs and symptoms of Paget's disease
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The first symptom of Paget's disease is usually a scaly, red rash affecting the nipple and sometimes the dark area of skin surrounding the nipple (the areola). The rash always affects the nipple first and may then affect the areola. It doesn't go away and may become sore.
The skin of the nipple and areola may be inflamed. There may also be crusting, bleeding and ulceration. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast.
Around half of all women who have Paget’s disease have a breast lump that can be felt at the time the disease is diagnosed.
How Paget's disease is diagnosed
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Paget’s disease can be confused with other skin conditions such as eczema, dermatitis and psoriasis, because they can look very similar. This can make Paget’s disease difficult to diagnose.
Paget’s disease usually affects the nipple first and then the surrounding tissue, whereas other skin conditions usually affect the areola (the dark area of skin around the nipple) first, and then spread into the nipple.
Several tests may be carried out to diagnose Paget’s disease of the breast. They may include the following.
Mammogram (breast x-ray)
Mammograms may be used to look for changes in the affected breast and to check the other breast.
A gel is spread on the breast and a small device that emits sound waves is rubbed over the area. A computer then converts these sound waves into a picture of the breast tissue. This test is painless and takes just a few minutes.
A gel is spread on the breast and a small device that emits sound waves is rubbed over the area. The echoes are converted into a picture of the breast tissue by a computer. This test is painless and takes just a few minutes.
This is the main test for cancer cells below the skin surface. A small sample of skin and underlying breast tissue is taken and sent to the laboratory to be examined under a microscope. A local anaesthetic is usually given to numb the area first. The biopsy may be taken at the same time as an ultrasound to ensure that it's taken from the correct area.
Imprint or scrape cytology
Cells from the affected area can be scraped, or pressed, onto a glass slide, to be examined under a microscope.
Treatment for Paget's disease
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Surgery is the main treatment for Paget’s disease. Other treatment depends on whether the underlying breast cancer is DCIS or an invasive cancer. Some people don't need any further treatment after surgery. Others may have a combination of treatments, including, radiotherapy, hormonal therapy, chemotherapy or a drug called trastuzumab (Herceptin®).
The type of operation you have will depend on how much the breast is affected. Your surgeon and breast care nurse will explain more about the type of operation that is best for your individual situation.
Usually an operation to remove all of the breast (a mastectomy) is done if the cancer affects a wide area, or if there's DCSI in different parts of the breast. Some women may have a new breast shape formed (breast reconstruction) at the same time as the mastectomy. But this may not be suitable for everyone, and breast reconstruction can be done later when treatment is finished.
Some women may have an operation to remove the affected breast tissue along with some surrounding normal tissue and also the nipple and areola (called breast-conserving surgery). This may be possible if there's only a small area of cancer and the surgeon thinks the appearence of your breast will still be good after surgery.
If you have an invasive cancer, your breast surgery will usually include removing some or all of the lymph nodes in your armpit.
Radiotherapy treats cancer by using high energy x-rays to destroy the cancer cells, while doing as little harm as possible to the healthy cells.
You will usually have radiotherapy to the remaining breast tissue after breast-conserving surgery. It’s given to reduce the risk of the cancer coming back in the breast. If the cancer is invasive, some women who have a mastectomy may be given radiotherapy to the chest afterwards.
Radiotherapy is given as a series of short daily sessions, usually Monday-Friday, with a rest at weekends. Each treatment takes 10-15 minutes. A course of radiotherapy for breast cancer usually lasts three weeks and is given to you as an outpatient.
Your doctor or nurse will discuss the treatment and possible side effects with you. The skin in the treatment area may become red or sore, or if you are dark skinned, it may become darker. You’ll be given advice on how to look after your skin. You’re likely to feel tired during treatment and this may continue for a month or two after it. Get plenty of rest, but balance this with gentle, regular exercise, such as short walks, which will give you more energy.
Side effects usually gradually disappear once your treatment has finished. You can read more about radiotherapy in our section on radiotherapy.
Some breast cancer cells have oestrogen receptors on their surfaces. This is called oestrogen receptor positive (ER positive) breast cancer. Oestrogen is a hormone produced in the body. It can encourage some breast cancer cells to grow.
If you have ER positive breast cancer, you'll be prescribed hormonal therapy to reduce the risk of cancer coming back and to protect the other breast. You usually have it for up to five years. There are different types of hormonal therapy and they work in different ways. Some hormonal therapies reduce the amount of oestrogen in the body, while others prevent oestrogen from reaching the cancer cells.
Drugs such as tamoxifen, anastrozole, letrozole (Femara®) or exemestane (Aromasin®) may be used.
Your doctor or breast care nurse will give you more information about the hormonal therapy you'll be having and any possible side effects it may have.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It's not used to treat an underlying DCIS, but it may be given if the cancer is invasive.
You will have the chemotherapy drugs given by injection into a vein (intravenously) or as tablets. Chemotherapy into the vein is given as a session of treatment, usually over a few hours. This is followed by a rest period of a few weeks, which allows your body to recover from any side effects of the treatment.
Side effects of chemotherapy can often be well controlled by medicines and usually improve when treatment is over. Some of the side effects the drugs may cause are increased risk of infection, tiredness, feeling sick (nausea) and hair loss.
Always let your doctor or nurse know about any side effects you have. There are usually ways in which they can be controlled or improved.
Our section on chemotherapy discusses the treatment and its side effects in more detail. We also have information on individual chemotherapy drugs.
Herceptin is one of a new group of drugs called monoclonal antibodies. Monoclonal antibodies are sometimes called targeted therapies because they work by ‘targeting’ specific proteins (receptors) on the surface of cells.
Some breast cancer cells have too much of a protein called HER2 (human epidermal growth factor 2) on their surface. The extra HER2 receptors stimulate the cancer cells to divide and grow. Herceptin locks on to the HER2 protein. This blocks the receptor and stops the cells from dividing and growing.
Herceptin only works in women who have cancer with high levels of the HER2 protein (HER2 positive breast cancer), which you will be tested for. It can be used to treat early breast cancer or breast cancer that's spread (secondary breast cancer).
Herceptin can affect the way the heart works so you will have tests to check the health of your heart first.
Herceptin is usually given once every three weeks for a year. You may have it with chemotherapy or after chemotherapy has finished. It’s given as a drip (infusion) into a vein in your hand or arm. Side effects can include flu-like symptoms, diarrhoea and headaches, but these tend to be mild.
Research into treatments for breast cancer are ongoing. Cancer specialists use clinical trials to assess new treatments. Before any trial is allowed to take place, an ethics committee must approve it and agree that it's in the interest of patients.
You may be invited to take part in a clinical trial. Your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or to withdraw from a trial at any stage. You'll then receive the best standard treatment available.
You may have many different emotions, including anger, resentment, guilt, anxiety and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition.
Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists can give you information about counselling in your area.
You may find it helpful to talk things over with your doctor or nurse, or with one of our . Family members and close friends can also offer support.
This section has been compiled using information from a number of reliable sources, including:
National Institute for Health and Clinical Excellence (NICE). Breast cancer (early and locally advanced). February 2009.
National Institute for Health and Clinical Excellence (NICE). Breast cancer (advanced). February 2009.
Harris JR, Lippman ME, Morrow M, Osborne CJ. Diseases of the Breast. 4th edition. 2010. Lippincott Williams and Wilkins.
Winchester, et al. Breast cancer. 2nd edition. 2008. Saunders.
Thank you to Professor Stephen Johnston, Consultant Medical Oncologist, and all of the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.