Paget’s disease of the nipple, also known as Paget’s disease of the breast or mammary Paget’s disease, is a rare type of breast cancer that primarily affects the nipple and areola (area surrounding the nipple). This type of cancer is often associated with carcinomas, and often develops in people who have another form of breast cancer.
This type of cancer develops from Paget cells, which appear as large, round cells filled with cytoplasm (a gelatinous, clear, liquid that fills up cells). The function of these cells are still being researched, but are often indicative of a malignancy in the breast.
Paget’s disease of the breast is more common in women, and is often found in people over 50 years old. However, anyone can develop this disease, including men.
Treatment
If Paget’s disease of the breast is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for Paget’s disease of the breast may include:
- Surgery, potentially including:
- Breast conserving surgery/lumpectomy (removal of the nipple and some surrounding tissue – a potential option for patients with early stage).
- Mastectomy (removal of entire breast(s)).
- Lymphonodectomy (removal of affected lymph nodes).
- Chemotherapy.
- Radiation therapy.
- Hormone therapy.
- Photodynamic therapy.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia Treatment Options page.
Risk factors
While the cause of Paget’s disease of the breast remains unknown, the following factors may increase your risk of developing the disease:
- A personal history of breast conditions and/or breast cancer.
- A family history of breast, ovarian and/or bowel cancer.
- Certain genetic mutations, including BRCA1, BRCA2 and HER2.
- Exposure to radiation.
- Obesity.
- Postmenopausal hormone therapy.
- Drinking alcohol.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Early symptoms
The symptoms of Paget’s disease of the breast are often confused for dermatitis or eczema. These may include:
- Hard, lumpy, or crusty appearance of the nipple and/or areola.
- Redness of nipple and/or areola.
- Itchiness of nipple area.
- Nipple discharge and/or oozing.
- A lump in the breast.
- Tingling or burning in the nipple/areola.
- A flattened or inverted nipple.
Not everyone with the symptoms above will have cancer, but see your GP if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have Paget’s disease of the breast, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities. More specifically, the doctor will check both breasts and lymph nodes in your armpits to feel for any lumps or abnormalities.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), mammogram (x-ray), and breast ultrasound depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Biopsy
Once the location of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. This is can be done by a surface biopsy (glass slide used to gently scrape cells for testing), shave biopsy (razor-like tool used to remove top layer of skin), punch biopsy (circular tool removes a disk-shaped piece of tissue), or a wedge biopsy (a scalpel removes a small wedge of tissue).
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage Paget’s disease cancers have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.