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Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a rare skin adenocarcinoma (cancer starting in glandular tissue) that generally affects areas with abundant sweat glands, such as anogenital (areas surrounding the anus and genitals) or axillary (the armpit area) skin. 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 metastasis 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 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 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 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 classified 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 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.

Surgery

This procedure is the most common treatment for patients with EMPD. Surgery is most often used in patients who have a tumour that hasn’t metastasised (spread to other parts of the body). The procedure itself will vary depending on the location and size of the tumour, but usually involves removing the tumour and a margin of healthy tissue to help prevent recurrence. Your doctor may recommend Mohs micrographic surgery, which takes portions of the cancer in stages and is performed under local anaesthesia.

In many cases, surgery is used in conjunction with chemotherapy and radiation therapy due to the unpredictable nature of this disease.

Radiation therapy

Radiation therapy, or radiotherapy, uses controlled doses of radiation to damage or kill cancer cells. It can be administered internally or externally. This may be a satisfactory alternative if you are not a suitable candidate for surgery. This treatment can also be used in addition to surgery. In some cases, radiotherapy is recommended prior to surgery to shrink the tumour so it’s easier to surgically remove. It can also be recommended after surgery to reduce the risk of cancer recurrence in the future.

Chemotherapy

Chemotherapy uses drugs to kill or slow the growth of cancerous cells, while minimising the damage to healthy cells. It is usually administered directly to the veins (intravenously), however in some cases in can be administered orally. You may receive a single chemotherapy drug, or a combination, depending on your individual factors. It is usually done in cycles of treatment sessions and periods of rest and can last several months. Chemotherapy can be recommended for patients prior to surgery in order to shrink the tumour, making it easier to surgically remove. In some cases, it can also be recommended after surgery to reduce the risk of cancer recurrence, or if your tumour is advanced and has metastasised. 

Clinical trials

Clinical trials are research studies performed to test new treatments. They present the opportunity for people, particularly those with rare or complex cancers, to receive very new treatments which are not yet available otherwise. While these studies have the potential to develop more treatment options, the risk of side effects can be high and are not always known. These trials might involve targeted therapies (to target only the cancer cells), hormonal therapies or immunotherapies.  

Clinical trials are often conducted in phases: 

  • Phase 0: these trials are used to determine how the body will react to experimental drugs. They are given in very small doses in very few people. 
  • Phase I: these trials are used in slightly bigger groups (20-80 people) to determine the safety of the drug, as well as any potential side effects that may arise. 
  • Phase II: these trials are used in larger groups (several hundred people) to further evaluate the safety of the drug, as well as whether it is working as it should be. 
  • Phase III: these trials are used in groups of several hundred to several thousand people. They are used to monitor any adverse effects of the drug, as well as compare it to other conventional or experimental treatment methods. 
  • Phase IV: these studies test the drug in the general population to determine any long-term adverse effects, as well as potential usage in treatment of other conditions. 

Ask your doctors about the availability of clinical trials, including whether you are suitable, and any benefits, risks and potential complications that may arise.  

Palliative care

Palliative care aims to improve the patient’s quality of life and alleviate symptoms, without trying to cure the disease. This option is beneficial for patients with advanced EMPD at any stage of treatment, not just towards the end of life. It can also be used in conjunction with other treatment options, such as surgery, radiotherapy and chemotherapy, with the intention of relieving pain, alleviating symptoms and meeting your physical, emotional, cultural and spiritual needs.

Risk factors

Because of how rare EMPD is, there has been limited research into the risk factors of this disease.

Early 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 (biopsy). 

Imaging & blood tests

The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), and/or positron emission tomography (PET 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 blood test may be taken to assess 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.

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 stage, rate/depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. Generally, early-stage EMPD have good 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.

References

Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.