Extramammary Paget disease (EMPD) is a rare type of skin cancer that generally affects areas with abundant sweat glands, such as anogenital (areas surrounding the anusEntry to the back passage; bum. and genitals) or axillary (the armpit area) skin. It is primarily an adenocarcinomaa cancer that grows in gland tissue, which is cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems. originating in glandular tissue. EMPD is different that mammary Paget disease, which has a similar appearance, but is found around the nipple and areola on the breast, and may be an indication of an underlying breast cancer.
EMPD can be classified into two categories, based on where the cancer originated from. The first is primary EMPD, which originates from the skin. The second category is secondary EMPD, which is associated with the metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. of a primary adenocarcinoma originating somewhere else in the body.
EMPD is most commonly diagnosed in people over 50 and is more prevalent in females. However, this disease can be developed at any age in any gender.
Treatment
If EMPD is detected, it will be staged and graded based on size, metastasis and how the cancer cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be 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 lymphA clear fluid that moves through the body through the lymphatic system, carrying cells that fight infection. 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 has 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 localisedOnly to one area/place 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 classified 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:
- Grade
• A score that tells how quickly a tumour might spread and grow by looking at how the abnormal cells and tissue look under a microscope.
• Grade is not the same as stage.
• Grading is different depending on the type of cancer.
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 cancers. 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 course of treatment for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for EMPD may include:
- Surgery to remove as much of the tumour as possible. This may involve a Mohs Micrographic surgery, which removes portions of the cancer in stages.
- Radiation therapy.
- Chemotherapy
• a chemical drug treatment to kill or slow-growing cancer Cells
• these drugs are called cytotoxic drugs
. - Clinical trials.
- Palliative careLessening pain without curing the disease..
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
Because of how rare EMPD is, there has been limited research into the risk factors of this disease.
Symptoms
The symptoms of EMPD are often confused with other skin conditions, and as such is often misdiagnosed. The most common occurrence of EMPD is the vulva in women and on the scrotum or around the anus in men. Common symptoms of this disease are:
- Itchiness of the skin.
- Burning, pain, tenderness or irritation.
- Skin discolouration around the area.
- Crusting and/or scaling lesions of the skin.
- Weeping lesions of the skin.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
Your doctor will usually start with a physical examination, followed by imaging tests to check for metastasis or underlying cancer. However, EMPD can only be diagnosed with a tissue sample (biopsyTo take a small piece of body tissue and test it in a laboratory.).
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography scan.), 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 bloodhello test may be taken to assessTo measure, look at and learn from. your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.