Extraskeletal myxoid chondrosarcoma | Sarcoma UK
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Extraskeletal myxoid chondrosarcoma

Extraskeletal myxoid chondrosarcoma (EMC) is a very rare type of soft tissue sarcoma, which is a type of cancer. It:

  • can be found anywhere in the body – although it often occurs in the upper parts of the arms or legs
  • develops from soft tissue cells (unlike chondrosarcoma which develops from cartilage cells)
  • is mostly diagnosed in middle-aged adults
  • affects more males than females
  • is thought to occur when chromosomes in cells break and re-join in the wrong way. Researchers are still trying to find out why this happens and whether this causes EMC.


There are an average of 14 cases of EMC diagnosed every year in England.

EMC makes up 0.36% of all soft tissue sarcomas.

For more information on these statistics, you can visit our soft tissue sarcoma data hub.

Signs and symptoms

Symptoms can vary depending on the size and location of your tumour. The most common symptom is a lump or swelling, which:

  • is often under the skin of the arms or legs
  • is often accompanied by pain and tenderness
  • can sometimes resemble a bruise
  • can restrict movement if the lump is near a joint.


A specialist will diagnose EMC with tests. You might have:

  • a physical examination – looking at and feeling your lump
  • a scan – taking pictures of the inside of the body. Scans might include an ultrasound, x-ray, CT, MRI or PET scans
  • a biopsy – taking and testing a sample of the tumour. A doctor will look to see if the biopsy looks like EMC.

To read more about these scans and tests, click here.



The main treatment for EMC is surgery. If you have surgery, your surgeon will remove the tumour. They’ll also aim to take out an area of normal tissue too. Sometimes people call this ‘taking a margin’. They do this to make sure they remove all of the cancer.

If your tumour is in your arms or legs, your surgeon will perform ‘limb-sparing surgery’. They do this to make sure the limb can continue to work well.

If the cancer has spread, your surgeon may need to perform a partial or full amputation to stop the cancer – although this is very rare.



Radiotherapy is a treatment that uses radiation to kill cancer cells.

You might have it before or after surgery. When used before surgery, it aims to make the tumour smaller so that it’s easier to remove. When used after surgery, it aims to kill off any cancer cells left after your operation.



Chemotherapy is a treatment that uses medicine to kill cancer cells.

It’s sometimes used if:

  • you have a high risk of the cancer coming back
  • your tumour has spread to other parts of the body (also known as metastasis).

It’s not clear how well chemotherapy works for people with EMC. Some types of chemotherapy have shown to work well while others have not. You may be more likely to have surgery (with or without radiotherapy) as your main treatment.


Targeted and experimental therapies

Researchers are learning more about treatments called targeted therapies and immunotherapies.

Targeted therapies are drugs that find and attack cancer cells. They work by targeting the differences a cancer cell has from normal cells. They do this to stop them from growing and spreading.

Immunotherapy is a type of treatment that uses your immune system to find and attack cancer cells.

Clinical trials have found that some targeted therapies and immunotherapies work well for EMC. But more research is needed before these treatments can be approved.

You may be offered a chance to join a trial. These trials can find new ways to diagnose and treat cancer. Some studies also look at the care and well-being of patients. Your doctor can give you more information on opportunities to take part in a trial.

There are trials recruiting people with EMC. For more information, visit our Clinical Trials Hub.

After treatment

After treatment, you’ll have follow-up appointments for several years. Your sarcoma nurse will provide a schedule. The follow-ups will usually include:

  • a chance to discuss symptoms
  • an examination to look for any signs of the cancer returning
  • a chest x-ray to rule out any secondary cancers in the lungs

Living with

EMC can come back after treatment. If it reappears in the same area as the original tumour, it’s known as a local recurrence.

If the cancer does reappear, it’s important to get treated quickly. You may have surgery and/or radiotherapy. Your treatment plan will be determined by your doctor.

It’s also useful to check for recurrences by examining your body. Your doctor or nurse will tell you what to look for.

If you’re worried about your cancer returning, you can talk to your doctor or nurse. They might decide to bring your follow-up appointment forward.

What if my cancer spreads to another part of my body?

If your cancer returns, it can be accompanied by cancer in other parts of your body. This is called ‘metastasis’ or ‘secondary cancer’.

Some people are diagnosed with sarcoma because their metastases has been found before the first tumour. In people with EMC, these secondary cancers can appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.

Secondary cancers can also appear in the bone and lymph nodes. Treatment for secondary cancer can involve surgery or radiotherapy. It can also involve other treatment as needed.

If you have any questions or if you need to talk to someone, our Support Line team are here for you.


More information

A scientific review on the current management of EMC.

Last reviewed: 9 April 2024

Next review due: 9 April 2027


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