Epithelioid sarcoma (ES) is a rare, soft tissue sarcoma (bone and tissue 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.) that slowly develops over time. ES has a high potential for metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (the process where 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
break away from primary cancer and form tumours in other parts of the body) and recurrence.
There are two types of ES. The most common variant is distal ES, which develops in the ‘distal’ (away from the centre of the body) portions of the body, such as hands, fingers, feet and toes. This variant is most commonly found in young adults. The less common variant is the proximal ES, which develops in ‘proximal’ (near the centre of the body) portions of the body, such as pelvis and genital tracts. This type is usually much more aggressive and is mainly found in adults.
While ES is mainly diagnosed in young adults, it can also develop in infants, children and teenagers.
Treatment
If ES 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 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 a 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 ES may include:
- Surgery to remove as much of the tumour as possible. In rare cases, a partial amputation of the affected limb may be required.
- 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
While the exact cause of ES remains unknown, the following factors might increase the risk of a person developing this type of cancer.
- Exposure to radiation.
- Genetics.
- Immune system abnormalities.
- Lymphedema (swelling caused by lymph nodeA small lump or mass of tissue in your body.A small lump or mass of tissue in your body.A small lump or mass of tissue in your body. removal or damage from cancer treatment).
- Exposure to chemicals.
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
ES presents with very few symptoms, or may be diagnosed without any symptoms at all. As the tumour grows, symptoms may include:
- A noticeable lump or swelling. This is usually painless.
- Pain, if the tumour is situated on nerves or muscles.
Not everyone with the symptoms above will have cancer but see your GP if you are concerned.
Diagnosis/diagnosing
ES is generally difficult to diagnosisUsing medical test results, identify and name a disease and/or condition., and is often misdiagnosed as other conditions. If your doctor suspects you have ES, they will order a range of diagnostic tests.
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.), 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 biopsyTo take a small piece of body tissue and test it in a laboratory. to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.
