PAGETS DISEASE OF THE BREAST

Paget’s disease of the breast is an uncommon form of breast cancer that first shows as changes to the nipple.

It occurs in fewer than 5% of all women with breast cancer. Men can also get Paget’s disease but this is very rare.

Signs and symptoms

The most common sign is a red, scaly rash involving the nipple, which may spread to the areola.

The rash can feel itchy or you may have a burning sensation. The nipple may be inverted (pulled in) and there may also be some discharge.

The symptoms of Paget’s disease can look like other skin conditions such as eczema or psoriasis. However, there are differences. For example, Paget’s disease affects the nipple from the start while eczema affects the areola region first and only rarely affects the nipple.
Paget’s disease usually occurs in one breast, while other skin conditions usually affect both breasts.
Approximately half of patients with Paget’s disease will also have an underlying lump. Most of these will be invasive cancers, which means the cancer has the potential to spread outside the breast.
Where there is no lump, most will be non-invasive or in-situ cancers. This means that the cancer cells are inside the milk ducts and have not developed the ability to spread either within or outside the breast.

Diagnosis

Because Paget’s disease can look like other skin conditions, it can be difficult to make a diagnosis.
Once your GP has referred you to a specialist, certain tests may be done to help make the diagnosis. They include:

• Mammogram
  You will usually have a mammogram (breast x-ray) to check whether there is any underlying cancerous tissue in the breast.
• Scrape cytology
  This involves scraping some cells from the skin of the nipple. The cells are put onto a slide so that they can be looked at under a microscope.
• Imprint cytology
  In this case an area of the affected nipple is pressed onto a slide. The cells on the slide can then be examined under a microscope.
• Biopsy
  You will probably have a biopsy taken to confirm the diagnosis. This means taking a small piece of skin and breast tissue from the affected area so that they can be looked at under a microscope. This is known as an incisional biopsy and can be done under local anaesthetic.

Treatment

Surgery
As for most types of breast cancer, surgery will be the first treatment. The type of surgery will depend on whether or not you have a lump and the extent of the cancer.
If you have a lump or the cancer is widespread in the breast then you are likely to be offered a mastectomy.
If you don’t have a lump and the cancer is confined to a small area, a wide local excision including the nipple followed by radiotherapy may be suggested.
In some cases you may be given the choice, which means the surgeon thinks both types of surgery will offer the same benefits.

It is important to find out whether the cancer has spread to the lymph nodes in the axilla. This will help to find out if you need any further treatment such as chemotherapy.

This means either removing some of the lymph nodes (sampling) or removing all the nodes (clearance) or having a sentinel node biopsy.
Adjuvant treatment
Adjuvant treatments are treatments given in addition to surgery and include chemotherapy, radiotherapy and hormonal therapy.
The aim of adjuvant treatments is to reduce the risk of the cancer coming back either in the same breast (local recurrence) or elsewhere in the body.
If you have a wide local excision you will probably be offered radiotherapy to reduce the risk of the cancer coming back in the same breast. If you have a mastectomy you may not need radiotherapy.

In some cases chemotherapy is recommended, for example if the cancer has spread to the lymph nodes.

If your tumour is oestrogen receptor positive, which means it depends on the hormone oestrogen for growth, hormone therapy will usually be recommended.
Herceptin (a targeted therapy) may be offered if the invasive cancer is HER2



SOURCE:  BREAST CANCER CARE.