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Paget's disease

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This page tells you about a condition called Paget's disease of the breast. You can find information about

 

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Paget’s disease

Paget's disease is a rare disease that occurs sometimes alongside breast cancer. It is found in 1 or 2 out of every 100 breast cancers (1 to 2%). Paget's disease starts in the nipple or in the area of darker skin surrounding it (the areola). It usually first appears as a red, scaly rash. It can be itchy. It looks very similar to other skin conditions such as psoriasis or eczema and may be mistaken for these.

How Paget's disease is diagnosed

A breast surgeon takes a sample of the affected skin tissue (a biopsy) from the nipple and sends it for examination under a microscope. If the biopsy shows Paget's disease, you then have a breast X-ray (mammogram). In many cases, Paget's disease is a sign of breast cancer or ductal carcinoma in situ (DCIS). DCIS means that there are cancer cells but they are contained within the lining of the ducts or lobes of the breast. About half of the women with Paget's disease have a lump behind the nipple. In 9 out of 10 cases, this is an invasive breast cancer. 

How Paget's disease is treated

You have surgery to remove either the whole breast (a mastectomy) or just the affected area (wide local excision). If you have invasive breast cancer your doctor may offer you radiotherapy, chemotherapy, hormone therapy or biological therapy after your surgery.

 

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What Paget's disease is

Paget's disease is a rare disease that is associated with breast cancer. It is found in 1 or 2 out of every 100 breast cancers (1 to 2%).

Paget's disease starts in the nipple or in the area of darker skin surrounding it (the areola). It usually first appears as a red, scaly rash of the skin over the nipple and areola. It can be itchy. If it isn't treated, or if you scratch it, it can bleed, ulcerate and may scab over. It looks very similar to other skin conditions such as psoriasis or eczema. It is sometimes diagnosed quite late, and this may be because it is often first treated as eczema, before any cancer tests are done.

 

How Paget's disease is diagnosed

Paget's disease is diagnosed from a biopsy. A breast surgeon takes a sample of the affected skin tissue (a biopsy) from the nipple and sends it to the laboratory for examination under a microscope. If the biopsy shows Paget's disease, you then have a mammogram

In many cases, Paget's disease is a sign that there is a breast cancer in the breast tissues behind the nipple. About half the women diagnosed with Paget's disease have a lump behind the nipple. In 9 out of 10 cases, this is an invasive breast cancer.

An invasive breast cancer is present in some women with Paget's disease who have no lump. But most women with no lump have carcinoma in situ. This means that there are cancer cells in the biopsy, but they are completely contained within the lining of the breast ducts or lobes. This is not an invasive breast cancer and so there is no chance that the cancer cells have spread.

 

Treatment for Paget's disease

You have surgery to remove either the whole breast or just the affected area. Further treatment depends on whether your results show that you have an invasive breast cancer, or not.

 

If you have breast cancer

For some women, it is possible to just have the area containing the cancer removed, together with a border of healthy tissue. This is followed by a course of radiotherapy to the rest of the breast.

Removal of the whole breast (mastectomy) may be the only option if you have a large area affected by Paget's or there is an area of invasive breast cancer behind the nipple. 

With a cancer in the central area of the breast, your surgeon may not be able to leave you with a good breast shape if you have surgery just to remove the cancer and surrounding tissue. You may get a better appearance if you have the whole breast removed and then have surgery to make a new breast shape (breast reconstruction). 

When you have your surgery, the surgeon will take out some of the lymph nodes under your arm to see if they contain cancer cells.

If your breast cancer cells have oestrogen receptors your doctor will probably suggest that you have treatment with hormone therapy. This reduces the risk of the cancer coming back in the same breast (or the chest wall after mastectomy). It also lowers the risk of the cancer cells spreading anywhere else in the body. And it reduces the chance of getting a new cancer in the other breast.

Your doctor may suggest further treatment with chemotherapy if there is a significant risk that the cancer may come back. They may suggest this if

  • Cancer cells were found in your lymph nodes
  • You had a large breast tumour
  • Your cancer cells were high grade (grade 3)

Giving chemotherapy helps to lower the risk of the cancer coming back in the future.

 

If you have no lump

Women who have Paget's disease but no breast lump usually have abnormal cells called carcinoma in situ in the lining of the ducts or lobules of the breast. Removal of the breast mastectomy is the most common treatment because the abnormal cells could develop into an invasive breast cancer in the future if they are not treated. 

Your surgeon may also remove some lymph nodes to check for signs of cancer. In 9 out of 10 women (90%), there is no sign of cancer in the lymph nodes. In most cases, the surgery will be all the treatment you need.

If the area of abnormal cells is very small, you may be able to have just the Paget's removed, along with a border of healthy tissue around it. This may be all the treatment that you need. Or your doctor may advise that you have radiotherapy after your surgery to lower the risk that the Paget's will come back.

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Updated: 5 October 2012