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Paget's disease of the nipple

Introduction

Paget’s disease of the nipple, also called Paget’s disease of the breast, is a rare type of breast cancer. It appears as an itchy, red rash on the nipple. The rash may extend to the darker area of skin surrounding the nipple, called the areola, or it can cause an open sore or eczema-like symptoms on the nipple.

The term Paget’s disease of the nipple is used to distinguish the condition from Paget’s disease of bone, which occurs when the normal cycle of bone growth is disrupted, causing the bones to become weakened and deformed. See the Health topic on Paget’s disease of bone for more information about this condition.

For simplicity, the rest of this section will use the term Paget’s disease to refer to Paget’s disease of the nipple or breast.

How common is Paget’s disease?

According to Cancer Research UK, Paget’s disease is found in one or two out of every 100 cases of breast cancer.

In the UK, around 46,000 people are diagnosed with breast cancer every year. Most of these are women over 50 years of age who have reached the menopause.

Paget’s disease can affect men, but this is extremely rare.

Outlook

Paget’s disease is treated in the same way as breast cancer. The first step of treatment is usually surgery to remove all or part of the breast, including the nipple and areola. This may be followed by a combination of:

  • chemotherapy, when medication that prevents cancer cells from dividing and growing (cytotoxic medication) is used to destroy cancer cells
  • radiotherapy, when controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells
  • biological or hormone therapy, which can treat certain types of cancer 

The sooner breast cancer is diagnosed, the better the outcome is likely to be. There is a good chance of recovery if it is detected in its early stages. For this reason, it is vital that women check their breasts regularly for any changes and always get any changes examined by their GP.

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Symptoms

Paget’s disease of the nipple or breast affects the skin of your nipple and the darker area of skin around the nipple (areola). Paget’s disease usually only affects one nipple and causes red, dry and scaly skin, similar to the skin condition eczema. This may be accompanied by:

  • itchiness or a burning sensation
  • bleeding after itching 
  • sore and broken skin (ulceration), which may then scab over
  • discharge from the nipple, which may be streaked with blood

Breast cancer

Paget’s disease is usually found alongside invasive breast cancer. This is breast cancer that could potentially spread. In around half of all cases of Paget’s disease, you will also have a lump in your breast, often in the tissue behind the nipple.

Most breast lumps are found by the women who have them. It is very important to be aware of any lumps or changes in the appearance, feel or shape of your breasts. The sooner a cancerous lump is detected, the better the chance of treating it successfully.

Other signs of breast cancer include:

  • a lump or swelling in either of your armpits
  • a change in the size or shape of one or both of your breasts
  • dimpling on the skin of your breasts or the nipple becoming inverted 
  • bleeding from your nipple

See the Health topic on Breast cancer - symptoms for more information.

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Causes

Paget’s disease of the nipple or breast is usually a sign of breast cancer in the tissue behind the nipple. The breast cancer can either be:

  • invasive breast cancer, where the breast cancer cells have invaded the surrounding breast tissue
  • in situ breast cancer, where the breast cancer cells are contained in one or more areas of the breast but have no ability to spread

In around half of all cases of Paget’s disease, a lump is found in the breast. Nine out of 10 of those people with a lump will have invasive breast cancer. Of the people who do not have a lump, 4 out of 10 will have invasive breast cancer. The remaining people without a lump will have in situ breast cancer.

Risk factors for breast cancer

Several factors can increase your risk of developing breast cancer. These include:

  • Age: your risk of developing breast cancer increases as you get older.
  • Family history: having a close relative with breast cancer can increase your chance of developing the condition.
  • Being overweight: you can find out if you are overweight or obese with the Healthy weight calculator.
  • Alcohol: your risk of developing breast cancer can increase with the amount of alcohol you drink.

See the Health topic on Breast cancer - causes for more information about the risk factors and causes that have been linked to breast cancer.

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Diagnosis

See your GP if you notice:

  • any changes to the skin of your nipple or areola (darker area of skin around your nipple)
  • any changes to your breasts, particularly lumps

Paget’s disease of the nipple or breast can be confused with eczema, a skin condition that causes red, itchy and dry skin. See your GP for a diagnosis rather than assuming you have eczema. Paget’s disease is often a sign that you have breast cancer and the sooner this is diagnosed, the better the outcome is likely to be.

Your GP will examine both your breasts, even if you only have a problem in one of them. Your GP may also ask:

  • about your symptoms and how long you have had them
  • if you have a history of eczema or have eczema anywhere else on your body
  • if you have a personal or family history of breast cancer
  • your age and whether you have started the menopause yet (when a woman’s periods stop)
  • if you are taking any medication, including hormone replacement therapy, which is used to treat some of the symptoms of the menopause, or the oral contraceptive pill
  • how much alcohol you drink
  • how much you weigh and if you have recently gained weight

If your GP thinks you may have breast cancer, they will refer you to a specialist breast clinic for tests. 

Breast clinic

At the breast clinic, a specialist or breast nurse will carry out tests to determine whether you have breast cancer and what type of breast cancer you have. They may also take photographs of your breasts to keep a record of their current appearance and any further changes that may occur.

If you are diagnosed with breast cancer, the tests can also identify:

  • the stage of the cancer (how far the cancer has spread at the time of diagnosis)
  • the type of cancer and whether it is spreading

Your cancer specialist (oncologist) will discuss these with you in detail.

Determining the stage and grade of your breast cancer will help your doctors decide on the best treatment. However, the stage and grade alone cannot predict how your condition will progress. It also depends on factors such as:

  • the type of breast cancer
  • your overall health

Paget’s disease is usually first diagnosed with a skin biopsy. This is followed by further tests to identify the extent of your breast cancer.

Skin biopsy

A biopsy involves taking a sample of tissue, which is then examined under a microscope and tested to see if it is cancerous.

If you have Paget’s disease, you will then have a mammogram.

Mammogram

A mammogram is a simple procedure that uses X-rays to create an image of the inside of your breasts. It can identify early changes in your breast tissue, when it may be difficult to feel a lump.

Younger women usually have denser breasts in which changes are more difficult to identify, so a mammogram is not as effective in women under 35 years of age. In this case, your doctor may suggest that you have a breast ultrasound instead (see below).

If you need to have a mammogram, your radiographer (X-ray specialist) will position one of your breasts on a flat X-ray plate. A second X-ray plate will press down on your breast from above, so that it is temporarily compressed and flattened between the two plates. An X-ray will then be taken, which will give the clearest possible image of the inside of your breast.

The procedure will then be carried out on your other breast.

You may find the mammogram a bit uncomfortable or even painful, but it only takes a few minutes. Your doctor will examine the image produced by a mammogram for indications of cancer, such as areas of calcium within your breast tissue that show up on the X-ray (calcification).

Breast ultrasound

If you are under 35 years of age, you may need to have a breast ultrasound. This is because your breasts may be too dense for a mammogram. Your doctor may also suggest a breast ultrasound if they need to know whether a lump in your breast is solid or contains liquid.

Ultrasound uses high-frequency sound waves to produce an image of the inside of your breasts. An ultrasound probe or sensor will be placed over your breasts to create an image on a screen. The image will show any lumps or abnormalities that may be present in your breasts.

See the Health topic about Ultrasound scans for more information about this procedure.

Magnetic resonance imaging (MRI) scan

A magnetic resonance imaging (MRI) scan uses a strong magnetic field and radio waves to produce a detailed picture of the inside of your body. If necessary, an MRI scan can be used to highlight the areas in your body where cancer has spread. An MRI scan can also show in detail the exact size and shape of a lump inside your breast.

An MRI scan may be used if your mammogram or breast ultrasound do not show a lump in your breast in enough detail. See the Health topic about MRI scans for more information about his procedure.

Further tests

Other tests can be used to confirm the diagnosis and determine what types of treatment might be used. See Breast cancer - diagnosis for more information about these alternative tests.

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Treatment

Paget’s disease of the nipple or breast is often associated with other forms of breast cancer. It will be treated in the same way as more common types of breast cancer. The most common treatment for Paget’s disease is surgery to remove the whole breast, called a mastectomy (see below).

You will receive treatment from a team of healthcare professionals led by a cancer specialist (oncologist). Discuss any concerns you have with your oncologist, who will be able to explain each phase of your treatment.

Surgery

If you have Paget’s disease, surgery is often the first form of treatment you will receive. There are two types of surgery:

  • Mastectomy is surgery to remove the whole breast, which can be followed by reconstructive surgery to recreate the removed breast.
  • Breast-conserving surgery is surgery to remove just the cancerous lump (tumour) in your breast.

These forms of surgical treatment will also involve removing the nipple and the darker area of skin surrounding the nipple (areola).

The two different types of surgery are described in more detail below.

Mastectomy

A mastectomy removes all your breast tissue, including your nipple. You may need to have a mastectomy if the tumour is large or very central in your breast.

See the Health topic about Mastectomy for more information about this procedure, including the recovery process and possible complications.

If you have Paget’s disease, it is likely that the cancer is in the central part of your breast. This may mean that a mastectomy is your only option, as your surgeon will not be able to leave you with a very good breast shape if they try to remove only the central part of your breast.

The type of mastectomy you have will depend on whether the cancer has spread to your lymph nodes or to your chest wall muscles. Lymph nodes are small glands under your breast and in your armpit that filter bacteria from the body. It is more likely that your cancer has spread to the lymph nodes if you had a lump in your breast when you were diagnosed with Paget's disease.

If the cancer has not spread to your lymph nodes, you may have a simple mastectomy in which only your breast is removed. If the cancer has spread to your lymph nodes, you may need a modified or radical mastectomy, in which your breast is removed along with lymph nodes under your arm or your chest wall muscles.

If you have lymph nodes removed from your armpit during a mastectomy, the scarring can sometimes block the filtering action of the lymph nodes, causing a condition called lymphoedema. This is a long-term condition, but can be treated with:

  • massage
  • compression sleeves, which are tightly fitting bandages that push excess fluid out of your arm

Lymphoedema can develop months or sometimes years after surgery. See your breast care nurse or GP if you notice any swelling in your arm or hand on the side of your operation. See the Health topic about Lymphoedema for more information about this condition.

Breast-conserving surgery

The aim of breast-conserving surgery is to save as much of your breast as possible while removing as much of the cancer as possible to prevent it from recurring. You may be able to have breast-conserving surgery if the cancer is in its early stages or has not spread beyond your breast.

Breast-conserving surgery ranges from a lumpectomy or wide local excision, in which just the tumour and a little surrounding breast tissue is removed, to a partial mastectomy or quadrantectomy, in which up to a quarter of your breast is removed.

If you have Paget’s disease, your nipple and the darker area of skin around the nipple (areola) will be removed. You should be offered reconstruction surgery to improve the appearance of your breast after surgery (see below).

If you have breast-conserving surgery, the amount of breast tissue you have removed will depend on:

  • the type of cancer you have
  • the size of the tumour and where it is in your breast
  • the amount of surrounding tissue that needs to be removed

Your surgeon will always remove an area of healthy breast tissue around the cancer, which will be tested for traces of cancer. If there is no cancer present in the healthy tissue, there is less chance that the cancer will recur. If cancer cells are found in the surrounding tissue, you may need to have more tissue surgically removed from your breast.

After breast-conserving surgery, you may need to have treatment with radiation (radiotherapy) to destroy any remaining cancer cells.

Breast reconstruction

If you have a mastectomy, you may be able to have reconstructive surgery to recreate your breast. This can be done by:

  • inserting a breast implant
  • using tissue from another part of your body to create a new breast

You can decide whether to have reconstructive surgery after a mastectomy. Discuss your options fully with your surgeon and breast nurse before making a decision.

In some cases, it may be possible to have reconstructive surgery carried out at the same time as the mastectomy. In other cases, you may need to delay the surgery, for example if you need radiotherapy.

It may also be possible to have reconstructive surgery after breast-conserving surgery to improve the appearance of your breast and create a nipple.

Creating a nipple

A nipple can be created by:

  • tattooing a nipple on
  • using your own body tissue, such as tissue from your other nipple, although around half of these flatten out and shrink over time
  • using a stick-on latex (rubber) nipple, which can be made from a mould of your other nipple, so they are identical, and stuck on every day with glue and removed for washing

Prostheses

Women who decide against breast reconstruction can wear a false breast or breast prosthesis, which are available free on the NHS. After having a mastectomy, you may have a temporary fibre-filled prosthesis and a permanent prosthesis made from silicone, which can be replaced every two years.

Further treatment

After your surgery, you may need further treatment if you have invasive breast cancer, where the breast cancer cells have spread into other tissue in your breast. If you had in situ breast cancer, where the breast cancer cells were contained in one area of your breast, surgery may be all the treatment you need.

Other types of treatment for breast cancer include:

  • Chemotherapy: cytotoxic medication that prevents cancer cells from dividing and growing is used to destroy cancer cells.
  • Radiotherapy: controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells.
  • Biological therapy: if your breast cancer is HER2 positive, biological therapy can treat the cancer by stopping the effects of HER2 and helping your immune system to fight off cancer cells.
  • Hormone therapy: if your breast cancer is hormone-receptor positive, hormone therapy can treat the cancer by lowering the levels of hormones in your body or stopping their effects.

The amount and type of treatment you receive will depend on:

  • the type of breast cancer you have and whether it is spreading
  • the stage of the cancer, which indicates how far the cancer has spread 
  • your overall health

See the Health topic on Breast cancer - treatment for more information about the different treatments available for breast cancer, including the medications used and the side effects of treatment.

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Prevention

Paget’s disease of the nipple or breast is a form of breast cancer.

A number of factors have been identified that may make the chances of developing breast cancer less likely. See the Health topic on Breast Cancer - prevention for more information about these.

There are also methods of screening for breast cancer. Screening is a way of identifying apparently healthy people who may be at increased risk of a condition. For breast cancer, screening is carried out with mammograms, a procedure that uses X-rays to create an image of the inside of your breasts.

Screening

The NHS Breast Screening Programme provides free breast screening every three years for all women in the UK who are 50 years of age or over.  Women over age 70 may ask us for an appointment every three years by getting in touch with their local Breast Test Wales screening centre.

NHS breast screening is not usually available for younger women because mammograms are not as effective on younger breasts. If you are below the screening age and worried about changes in your breasts or have a family history of breast cancer, speak to your GP.

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