1. What is Paget’s disease of the breast?
2. What are the symptoms of Paget’s disease?
3. Paget’s disease and DCIS
4. Can Paget’s disease be invasive?
5. How is Paget’s disease diagnosed?
6. How is Paget’s disease treated?
7. Further support
Paget’s disease of the breast is an uncommon type of breast cancer that usually first shows as changes to the nipple. It occurs in less than 5% of all women with breast cancer. Men can also get Paget’s disease but this is very rare.
Paget’s disease of the breast is not the same as Paget’s disease of the bone.
The most common symptom is a red, scaly rash on the nipple, which may spread to the darker area of skin around the nipple (areola). The rash can feel itchy or you may have a burning sensation. The nipple may be pulled in (inverted). There may also be some liquid (discharge) coming from the nipple.
Is it Paget’s disease or eczema?
Paget’s disease can look like other skin conditions such as eczema or psoriasis. But there are differences. For example, Paget’s disease affects the nipple from the start while eczema generally affects the areola and only rarely affects the nipple. Also, Paget’s disease usually occurs in one breast while most other skin conditions tend to affect both breasts.
It’s important to stay breast aware and if you notice a change in either breast tell your GP as soon as possible.
Most people with Paget’s disease will have an early form of breast cancer – known as ductal carcinoma in situ (DCIS) – somewhere in the breast.
DCIS is graded as either low, intermediate, or high grade, based on what the cells look like under the microscope. With Paget’s disease it’s likely to be high-grade DCIS.
DCIS means that cancer cells have developed inside the milk ducts, but remain entirely in situ (in their place of origin). They have not yet developed the ability to spread outside the ducts, either into the surrounding breast tissue or to other parts of the body.
Because it’s confined to the breast ducts, if treated DCIS has a good outlook (prognosis). However, if DCIS is left untreated, the cancer cells may develop the ability to spread from the ducts into the surrounding breast tissue and become an invasive breast cancer (see below). Although the type, size and grade of the DCIS can help predict if it will become invasive, there is currently no way of knowing for certain if this will happen.
Some people with Paget’s disease will also have developed an invasive breast cancer. In many cases this will mean a breast lump has developed. Invasive breast cancer is breast cancer that has the potential to spread from the breast to other parts of the body. Even when there is no lump, some people may still have an invasive cancer.
Because Paget’s disease is rare and can look like other skin conditions, it’s not always diagnosed straight away. Once your GP has referred you to a specialist, you may have several tests, including:
- a mammogram (a breast x-ray)
- an ultrasound scan (uses high-frequency sound waves to produce an image)
You will usually then have a biopsy to confirm the diagnosis. A biopsy is the removal of tissue to be looked at under a microscope. The kind of biopsy you have will depend on your symptoms. For example:
- a nipple scrape removes cells from the skin of the affected nipple
- a punch biopsy removes a small circle of tissue from the skin of the breast or nipple.
- a core biopsy removes a small sample of tissue from the area of concern if this can be felt within the breast
If the area of concern can only be seen on a mammogram or ultrasound, you may have an image-guided biopsy. This is where samples of breast tissue are taken using a mammogram or ultrasound to help locate the exact position of the area of concern.
These tests can be done using a local anaesthetic. The samples of tissue or cells are sent to a laboratory where they are examined under a microscope to make a diagnosis. Find out more about the tests and procedures you may be having.Back to top
Surgery is usually the first treatment for Paget’s disease. The type of surgery will depend on the area of the breast affected, the size of the cancer compared to the size of your breast, and whether more than one area in the breast is affected.
You may be offered breast-conserving surgery (also called wide local excision or lumpectomy). This is the removal of the cancer with a margin (border) of normal breast tissue around it. For Paget’s disease, this type of surgery also includes the removal of the nipple and areola (the darker area of skin around the nipple).
A mastectomy (removal of all the breast tissue including the nipple area) is usually recommended if:
- the breast cancer affects a large area of the breast
- it hasn’t been possible to get a clear margin of normal tissue around the breast cancer using breast-conserving surgery
- there is more than one area of cancer in the breast
- breast-conserving surgery is not expected to provide an acceptable cosmetic result because of the position or size of the cancer
You may be offered a choice between a mastectomy and breast-conserving surgery depending on the size and location of the breast cancer within the breast. Your breast surgeon will discuss this with you and you can talk through your decision with your breast care nurse.
Breast reconstruction after Paget’s disease
If you are going to have a mastectomy, you will usually be given the option of having breast reconstruction to create a new breast shape, either at the same time as your mastectomy (immediate reconstruction) or months or years later (delayed reconstruction).
It is possible to have the nipple reconstructed after surgery for Paget’s disease of the breast, whether you have breast-conserving surgery or a mastectomy.
Choosing whether or not to have breast reconstruction is a very personal decision. Some women feel reconstruction is necessary to restore their confidence. Others prefer to wear an external breast form (prosthesis), and some women choose not to have reconstruction and not to wear a prosthesis.
Lymph node removal
If you have invasive breast cancer underlying the Paget’s disease, your specialist team will want to check if any of the lymph nodes (glands) under the arm (axilla) contain cancer cells. This, along with other information about your breast cancer, helps them decide whether or not you will benefit from any additional treatment after surgery. To do this, your surgeon is likely to recommend an operation to remove either some of the lymph nodes (a lymph node sample or biopsy) or all of them (a lymph node clearance).
Sentinel lymph node biopsy
Sentinel lymph node biopsy is widely used if tests before surgery show no evidence of the lymph nodes containing cancer cells. It identifies whether the sentinel lymph node (the first lymph node that the cancer cells are most likely to spread to) is clear of cancer cells. There may be more than one sentinel lymph node. If clear, this usually means the other nodes are clear too, so no more will need to be removed. Sentinel lymph node biopsy is usually carried out at the same time as your cancer surgery but may be done before your surgery.
If the results of the sentinel lymph node biopsy show that the first node or nodes are affected, more surgery or radiotherapy to the remaining lymph nodes may be recommended.
Sentinel lymph node biopsy is not suitable if tests before your operation show that your lymph nodes contain cancer cells. In this case it is likely that your surgeon will recommend a lymph node clearance.
Usually the lymph nodes under the arm don’t need to be removed if you have DCIS. This is because the cancer cells haven’t developed the ability to spread outside the ducts into the surrounding breast tissue. However, surgery to the lymph nodes may be recommended for some people with DCIS.
Find out more about surgery to the lymph nodes.
What are the adjuvant (additional) treatments?
After surgery, you may need further treatment. This is called adjuvant (additional) treatment and can include:
The aim of these treatments is to reduce the risk of breast cancer returning in the same breast or developing in the opposite breast, or spreading somewhere else in the body. Some of these treatments may be given before surgery. This is known as neo-adjuvant or primary treatment.
Whether chemotherapy is recommended will depend on various features of the cancer, such as its grade, size and whether the lymph nodes contain cancer cells. Chemotherapy may be used if the underlying breast cancer is invasive. It is not used to treat DCIS.
Radiotherapy uses high energy x-rays to destroy cancer cells. If you have breast-conserving surgery you will usually be offered radiotherapy to the breast to reduce the risk of the cancer coming back in the same breast.
Sometimes you may be offered radiotherapy to the nodes under your arm. Radiotherapy is sometimes given to the chest wall following a mastectomy, for example if some of the lymph nodes under the arm are affected.
The hormone oestrogen can stimulate some breast cancers to grow. A number of hormone therapies work in different ways to block the effect of oestrogen on cancer cells.
Hormone therapy will only be prescribed if your breast cancer has receptors within the cell that bind to the hormone oestrogen, known as oestrogen receptor positive or ER+ breast cancer. When oestrogen binds to these receptors, it can stimulate the cancer to grow.
All invasive breast cancers are tested for oestrogen receptors using tissue from a biopsy or after surgery. DCIS may be tested but this is not done in all hospitals.
If your cancer is oestrogen receptor positive, your specialist will discuss with you which hormone therapy they think is most appropriate.
Tests will also be done for progesterone (another hormone) receptor. The benefits of hormone therapy are less clear for people whose breast cancer is only progesterone receptor positive (PR+ and ER-). Very few breast cancers fall into this category. However, if this is the case for you your specialist will discuss with you whether hormone therapy is appropriate.
If your cancer is found to be hormone receptor negative, then hormone therapy will not be of any benefit to you.
This is a group of drugs that block the growth and spread of cancer. Targeted therapies target and interfere with processes in the cells that cause cancer to grow.
The most widely used targeted therapy is trastuzumab. Only people whose invasive cancer has high levels of HER2 (called HER2 positive) will benefit from having trastuzumab. HER2 is a protein that makes cancer cells grow.
There are various tests to measure HER2 levels which are done on breast tissue removed during a biopsy or surgery. If your cancer is found to be HER2 negative, then trastuzumab will not be of benefit to you.
Bisphosphonates are a group of drugs that can reduce the risk of breast cancer spreading in postmenopausal women. They can be used regardless of whether the menopause happened naturally or because of breast cancer treatment.
Bisphosphonates can also slow down or prevent bone damage. They’re often given to people who have, or are at risk of, osteoporosis (when bones lose their strength and become more likely to break).
Bisphosphonates can be given as a tablet (orally) or into a vein (intravenously).
Your specialist team can tell you if bisphosphonate treatment would be suitable for you.
Being diagnosed with breast cancer can make you feel lonely or isolated. There are people who can support you so don’t be afraid to ask for help if you need it.
Some people find it helpful to discuss their feelings and concerns with their breast care nurse or specialist. If you’d like to talk through your feelings and concerns in more depth over a period of time, you may want to see a counsellor or psychologist. Your breast care nurse, specialist or GP can arrange this.
Many people find it helps to talk to someone who has been through the same experience as them. Breast Cancer Now’s Someone Like Me service can put you in touch with someone who has had a diagnosis of breast cancer, so you can talk through your worries and share experiences over the phone or by email. You can also visit our confidential online Forum and join one of the ongoing discussions.
If you would like any further information and support about breast cancer or just want to talk things through, you can speak to one of our experts by calling our free Helpline on 0808 800 6000.