What is Paget's Disease?
Paget’s disease of the breast is a form of breast cancer that affects 1-2% of women who are diagnosed with the disease. It is usually recognised early because it causes obvious changes to the skin of the nipple. These can look a bit like eczema and although women may not immediately think that something is seriously wrong, they do tend to consult their doctor to get treatment for the ‘eczema’ that is troubling them.
The breast cancer is then identified, usually when it is still enclosed within the breast ducts. For this reason, Paget’s disease of the breast is also known as ductal carcinoma in situ. This literally means cancer in the breast ducts, which is localised and hasn’t spread.
What Does Paget’s Disease of the Breast Look Like?Women often confuse it for eczema because it starts as a red, sore rash that produces lots of dry skin and scales. It is usually noticed on the nipple or the areola, the darker area of thin skin that is just around the nipple. The skin can become very itchy, or it can burn and weeping and ulceration of the nipple is not uncommon. The nipple feels sore and swollen and the nipple itself can invert, but women rarely notice a breast lump. When they are examined, however, about 50% of women with these symptoms do have a lump just behind the nipple: that is the growing breast tumour.
Diagnosis and TreatmentUsually, GPs and nurse practitioners will be very alert to the possibility of breast cancer in a woman showing eczema like changes to the breast, particularly if the problem starts with the nipple. When the problem is caused by eczema, the skin becomes affected around the areola first and then the nipple becomes involved.
Apart from a clinical examination, it is then usual for the GP to arrange a mammogram and perhaps an ultrasound scan. If these show the definite presence of a lump, a biopsy will be done by fine needle aspiration. This takes a few of the cells away from the sore area, aiming to get inside the lump. These cells are then looked at by a histology specialist who can tell whether the cells have become cancerous.
The first line of treatment for Paget’s disease is surgery. An operation will either remove the whole breast, or it will just remove the front part. Unfortunately, with this type of breast cancer it is inevitable that a woman will lose the entire nipple, but breast reconstruction surgery done later can build up a reconstructed nipple. Techniques have advanced a great deal in the last few years and breast reconstruction is now offered to all women who have had breast surgery.
Follow up Treatment to Stop the Cancer RecurringAs the breast cancer in Paget’s disease is very localised, removing the affected breast tissue with a margin of healthy tissue is often curative. Women who have a mastectomy may not need radiotherapy afterwards, but this is usually recommended for women who have breast conserving surgery,
At the time of diagnosis, the histology specialist will do tests to show if the breast cancer has hormone receptors or HER-2 receptors. A tumour that has oestrogen receptors will have a much lower chance of recurrence if follow up hormonal therapy reduces the amount of oestrogen in the body. The main anti-oestrogen therapies of Tamoxifen and the aromatase inhibitors are often used after surgery for Paget’s disease. Herceptin treatment is used in women whose cancers are also HER-2 positive.
If your cancer was still localised at the time of surgery, you are unlikely to be offered chemotherapy but if the cancer cells have begun to spread to the lymph nodes or to elsewhere in the body, several cycles of chemotherapy may be recommended.