Paget's disease of the nipple

Overview

Paget's disease of the nipple, also known as Paget's disease of the breast, is a rare condition associated with breast cancer.

It causes eczema-like changes to the skin of the nipple and the area of darker skin surrounding the nipple (areola). It's usually a sign of breast cancer in the tissue behind the nipple.

About 1 to 4% of women with breast cancer have Paget's disease of the nipple. It can also affect men, but this is extremely rare.

The term Paget's disease of the nipple is used to distinguish the condition from Paget's disease of the bone (where bones become weak and deformed).

Symptoms of Paget's disease of the nipple

Paget's disease of the nipple always starts in the nipple and may extend to the areola. It appears as a red, scaly rash on the skin of the nipple and areola.

The affected skin is often sore and inflamed, and it can be itchy or cause a burning sensation. The nipple can sometimes be ulcerated.

The rash is often similar in appearance to other skin conditions, such as eczema or psoriasis. If you scratch it, or if it's left untreated, it can bleed, become ulcerated or scab over.

If you're experiencing itchiness, burning or bleeding but the nipple looks normal and is not red or scaly, it's unlikely to be Paget's disease of the nipple. However, you should still have it checked by a doctor.

Around half of all women diagnosed with Paget's disease of the nipple have a lump behind the nipple. In 9 out of 10 cases this is an invasive breast cancer.

Invasive cancer is where cancerous cells invade the surrounding breast tissue. Some women with Paget's disease have invasive breast cancer but do not have a lump.

However, most women with Paget's disease who do not have a lump have non-invasive cancer.

This is where the cancerous cells are contained in 1 or more areas of the breast and have not spread.

Causes of Paget's disease of the nipple

The cause of Paget's disease of the nipple is unknown. Some women appear to be more at risk of developing breast cancer, but not specifically Paget's disease. There are no known causes that lead to a specific type of breast cancer.

In terms of risk factors, you're more likely to develop breast cancer if you:

are older - the risk of developing breast cancer increases with age
have close relatives with breast cancer
have had breast cancer before
have had a type of benign (non-cancerous) breast disease before - certain types of benign breast disease may be associated with an increased risk of breast cancer, but this is only seen in a very small number of women
are overweight - particularly if you've been through the menopause (you can use the BMI calculator to check if you're a healthy weight)
drink a lot of alcohol - your risk can increase with the amount of alcohol you drink

Diagnosing Paget's disease of the nipple

You should see your GP if you notice any changes in the skin of your nipple or areola.

As Paget's disease of the nipple is associated with breast cancer, the sooner it's diagnosed, the better the outcome is likely to be.

Also see your GP if you develop a lump in your breast. Although most breast lumps are not cancerous, it's important to have them checked out.

A biopsy is used to confirm a suspected diagnosis of Paget's disease of the nipple. A small tissue sample (punch biopsy of the skin) will be taken from your nipple and examined under a microscope to see if it's cancerous.

If the results of the biopsy indicate Paget's disease, you'll have a mammogram (a low-dose breast X-ray) to investigate further.

Treating Paget's disease of the nipple

Surgery is the main treatment for Paget's disease of the nipple. Depending on whether the cancer has spread, surgery will either involve removing the whole breast (a mastectomy), or the nipple and areola with the breast tissue underneath them (a central excision).

If the whole of your breast is removed, breast reconstruction surgery can be used to create a breast shape to match your remaining breast.

You may also need further treatment if you have invasive breast cancer. This may be a combination of:

chemotherapy - where powerful medicine is used to destroy cancerous cells
radiotherapy - where controlled doses of high-energy radiation are used to destroy cancerous cells
targeted or hormone therapy - which lowers the risk of cancer returning in the same breast (and affecting the other breast), and it lowers the risk of cancerous cells spreading elsewhere in the body

If Paget's disease is detected and treated in its early stages, there's a good chance of a full recovery.

Reducing the risk of breast cancer, including Paget's disease of the nipple

Modifying certain lifestyle factors, such as reducing the amount of alcohol you drink and making sure you exercise regularly, may reduce your risk of developing certain types of cancer, including breast cancer.

Breast cancer screening is also used to help detect breast cancer early. In the UK, the NHS Breast Screening Programme provides free breast screening every 3 years for all women aged 50 to 70.

In some areas, women aged 47 to 49 and 71 to 73 are also invited for screening as part of a study looking at whether the age range for breast screening should be extended.

Diagnosis

As Paget's disease of the nipple is usually a sign of breast cancer, it's very important you see your GP if you notice changes to the tissue or skin of your breast.

In particular, you should tell your GP if you notice any changes to:

the skin of your nipple or areola (the darker area of skin around the nipple)
your breasts, particularly lumps in your breast

Paget's disease of the nipple can sometimes be confused with eczema, a skin condition that also causes red, itchy and dry skin.

Therefore, you should visit your GP for a diagnosis rather than assuming you have eczema. Paget's disease is a form of breast cancer and the sooner it's diagnosed, the better the outcome is likely to be.

Examination and history

Your GP will examine both breasts, even if you only have a problem with 1 of them. They may also ask you:

about your symptoms and how long you've had them
whether you've had breast cancer or have a family history of it
your age and whether you've had the menopause (when a woman's monthly periods stop)
whether you're taking any medication, including hormone replacement therapy (HRT), 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've recently gained weight

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

Breast clinic

At the breast clinic you'll have tests to find out whether you have breast cancer and, if you have, what type of breast cancer.

Staff at the clinic may photograph your breasts to record their current appearance and help identify any further changes that may occur.

Tests you might have at the clinic may include:

an examination of your breasts to check for lumps or other abnormalities
a mammogram
an ultrasound scan - which is the first line of investigation used in younger women, and those who are pregnant or lactating
a skin biopsy will be carried out if Paget's disease is suspected (a punch biopsy of the skin of the nipple and/or areola)

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 often have denser breasts than older women, which can make identifying changes more difficult. Therefore, mammograms are not as effective in women under 35 years of age. If you're under 35, your doctor may suggest you have a breast ultrasound instead (see below).

However, if Paget's disease of the nipple is confirmed, mammography will become an important part of pre-surgery assessment.

During a mammogram, the radiographer will position 1 of your breasts on a flat X-ray plate. A second X-ray plate will press down on your breast from above, temporarily compressing and flattening it between the 2 plates.

An X-ray will be taken, which will give a clear image of the inside of your breast. The procedure will then be carried out on your other breast.

Having a mammogram can be slightly uncomfortable or even painful, but it only takes a few minutes. The image that's produced will be looked at by 2 people, called film readers, image readers or radiologists, for signs of cancer.

Breast ultrasound

If you're under 35 years of age, a breast ultrasound may be recommended instead of a mammogram. This is because your breast tissue may be too dense for a mammogram. Your doctor may also suggest a breast ultrasound if they need to find out whether a lump in your breast is solid or contains liquid.

You may also have an ultrasound scan if you're pregnant or lactating.

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 are present.

Skin biopsy

A skin biopsy is often used to confirm a diagnosis of Paget's disease of the nipple. A small tissue sample will be taken from your nipple or the skin around it. The sample will be examined under a microscope and tested to see if it's cancerous.

Further tests

If a diagnosis of breast cancer is confirmed, a number of other tests can be carried out to help determine what types of treatment might be used.

Treatment

Paget's disease of the nipple is usually associated with breast cancer.

It's treated by removing the cancerous part of the breast, or sometimes the entire breast using a procedure called a mastectomy.

You can discuss any concerns you have with your oncologist (cancer specialist), who will be able to explain each phase of your treatment.

Surgery

If you're diagnosed with Paget's disease of the nipple, surgery is often the first type of treatment you'll receive.

The 2 main types of surgery are:

mastectomy - surgery to remove the whole breast, which can be followed by reconstructive surgery to recreate the removed breast
breast-conserving surgery - where only the cancerous lump (tumour) and a little surrounding breast tissue are removed

These types of surgery will also involve removing your nipple and the darker area of skin surrounding it (the areola).

The 2 different types of surgery are discussed in more detail below.

Mastectomy

During a mastectomy, all of your breast tissue, including your nipple, will be removed. You may need to have a mastectomy if:

the tumour is large or in the centre of your breast
there's more than 1 area of breast cancer
breast-conserving surgery will not provide acceptable results

If lymph nodes (small glands) are removed from your armpit during a mastectomy, the scarring may block the filtering action of the lymph nodes, resulting in a condition called lymphoedema.

This is a long-term condition, but can be treated with:

specialised massage techniques
compression sleeves - tightly fitting bandages that push excess fluid out of your arm
exercises
skin care

Lymphoedema can develop months or sometimes years after surgery. Contact your GP or breast care nurse if you notice any swelling in your arm or hand on the side of your operation.

Further treatment

After your surgery, you may need further treatment if you have invasive breast cancer (where the cancerous cells have spread into other tissue in your breast).

If you have non-invasive breast cancer (where the breast cancer cells are contained in 1 area of your breast), surgery may be the only treatment you need.

Other types of treatment for breast cancer include:

chemotherapy - where powerful medicine is used to destroy cancer cells and prevent them dividing and growing
radiotherapy - where controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells
targeted therapy - if your breast cancer is HER2 positive, targeted therapy, usually a medicine called trastuzumab, can be used to treat the cancer by stopping the effects of HER2 and helping your immune system fight off cancer cells
hormone therapy - if your breast cancer is hormone-receptor positive, hormone therapy can be used to treat the cancer by lowering the levels of hormones in your body or stopping their effects

Prevention

A number of things may help reduce your risk of developing breast cancer.

Diet and lifestyle

Exercising regularly and eating a healthy, balanced diet are known to help prevent many forms of cancer, as well as other serious health conditions, such as heart disease and diabetes.

Studies have looked at the link between breast cancer and diet and, although there are no definite conclusions at the moment, there are benefits for women who maintain a healthy weight, take regular exercise and who have a low intake of saturated fat and alcohol.

It's been suggested that exercising regularly (a minimum of 150 minutes or 2 hours 30 minutes a week) can reduce your risk of developing breast cancer by up to a third.

If you've experienced the menopause (when your monthly periods stop), it's very important you're not overweight or obese. This is because these conditions cause more oestrogen to be produced, which can increase the risk of breast cancer.

Breastfeeding

Studies have shown that women who breastfeed are statistically less likely to develop breast cancer than those who do not.

The reasons for this are not fully understood, but it could be because women do not ovulate as regularly while they're breastfeeding and their oestrogen levels remain stable.

Medicine

Medicines called tamoxifen and raloxifene are available on the NHS for women with an increased risk of developing breast cancer. They are usually only given by breast cancer specialists.

These medicines may not be suitable if you've had blood clots or womb cancer in the past, or if you have an increased risk of developing these problems in the future. If you've already had a mastectomy to remove both breasts you will not be offered these medicines because your risk of developing breast cancer is very small.

Either tamoxifen or raloxifene can be used in women who've had the menopause.

A course of treatment with tamoxifen or raloxifene will usually involve taking a tablet every day for 5 years.

Raloxifene can cause side effects including flu-like symptoms, hot flushes and leg cramps. Side effects of tamoxifen can include hot flushes and sweats, changes to your periods and nausea and vomiting.

Tamoxifen and raloxifene can increase your risk of developing blood clots, so you should stop taking them before any planned surgery.

Raloxifene is not currently licensed for reducing the chances of getting breast cancer in women with an increased risk of developing it. However, it can still be given by a specialist.

Breast screening

Breast screening can pick up breast cancer before it forms a lump. The procedure uses mammograms, where X-rays are taken, to create an image of the inside of your breasts.

The NHS Breast Screening Programme provides free breast screening every 3 years for all women in the UK who are 50 years to 70. In some areas, women aged 47 to 49 and 71 to 73 are also invited for screening as part of a trial looking at whether the screening age range should be extended.

NHS breast screening is not usually available for women under 47 years of age. This is because younger women tend to have denser breast tissue, which makes mammograms less effecitve at identifying abnormalities.

Speak to your GP if you're below screening age and worried about changes in your breasts, or you have a family history of breast cancer.

The information on this page has been adapted by NHS Wales from original content supplied by

NHS website nhs.uk