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 areolaarea of pigmented skin surrounding the nipple. This type of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs is often associated with carcinomas, and often develops in people who have another form of breast cancer.
This type of cancer develops from Paget cellsthe basic structural and functional unit of all living things, 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, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Lumpectomya surgical procedure where the tumour and a small amount of surrounding breast tissue are removed, also known as breast conserving surgery, also known as breast-conserving surgerya surgical procedure where the tumour and a small amount of surrounding breast tissue are removed, also known as a lumpectomy (a potential option for patients with early stage).
- Mastectomycomplete or partial removal of one or both breasts.
- Lymphadenectomysurgical removal of lymph node(s).
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Hormone therapymedication that alters the levels of certain hormones in the body, such as oestrogen and progesterone.
- Photodynamic therapya procedure that involves inserting a light-sensitive drug (photosensitiser) to shrink and damage cancer cells when exposed to a light source.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
While the cause of Paget’s disease of the breast remains unknown, the following factors may increase your riskthe possibility that something bad will happen of developing the disease:
- A personal history of breast conditions and/or breast cancer.
- A family history of breast, ovarian and/or bowelportion of the digestive system that digests food (small bowel) and absorbs salts and water (large bowel); also called intestines cancer.
- Certain genetic mutations, including BRCA1, BRCA2 and HER2.
- Exposure to radiation.
- Obesity.
- Postmenopausal hormonea chemical substance produced by glands in the endocrine system that regulates various functions in the body 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.
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
If your doctor suspects you have Paget’s disease of the breast, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- Mammograma type of medical imaging that uses x-rays to create detailed images of breast tissue.
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
Some of the information regarding diagnosis was obtained from the Paget’s disease of the Breast page published by the National Cancer Institute.
