Desmoplastic small round cell tumour | Sarcoma UK
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Desmoplastic small round cell tumour

Desmoplastic small round cell tumour is a rare type of soft tissue sarcoma, which is a type of cancer.

It can occur anywhere throughout the body. But, it almost always occurs in the abdomen (tummy) and is very rare in other parts of the body.

It’s often associated with children and young adults up to the age of 30. It’s also much more common in males than females.

Sometimes, chromosomes in cells can break and re-join together in the wrong way. Some scientists think that this can cause desmoplastic small round cell tumour. Researchers are still trying to find out why this happens and whether this does cause it.


There are an average of 12 cases of desmoplastic small round cell tumour diagnosed every year in England.

Although it can affect anyone of any age, the median age at diagnosis is 23 years old.

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

Signs and symptoms

Symptoms of desmoplastic small round cell tumour can vary depending on the size and location of your tumour.

Before you are diagnosed, you may experience all, some or none of these symptoms:

  • A lump or swelling in the body, almost always in the abdomen
  • This lump can grow very quickly, and is often painful
  • Abdominal pain and swelling
  • Unexplained weight gain
  • Nausea
  • Difficulty urinating or constipation

If you have any of these symptoms, you should visit your GP.


A specialist doctor will diagnose desmoplastic small round cell tumour through a series of tests. These may include:

  • Physical examination – looking at and feeling any lump.
  • A scan – taking pictures of the inside of the body using scans such as CT or MRI scans.
  • A biopsy – taking and testing a sample of the tumour. A doctor will look to see if the biopsy looks like desmoplastic small round cell tumour and whether it contains the chromosome error.

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



The first treatment method for desmoplastic small round cell tumour is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – also called “taking a margin”. This is to make sure the surgeon removes all the cancer.


  • This treatment uses high-energy radiation to destroy cancer cells.
  • You may have radiotherapy either before or after surgery.
    • Before surgery, radiotherapy aims to shrink the tumour, making it easier to remove.
    • After surgery, radiotherapy aims to kill any cancer cells that surgery didn’t remove.


This treatment uses anti-cancer drugs to destroy cancer cells. Doctors may use it if you can’t have surgery or if you have a high risk of cancer returning. They might also use it if your cancer has spread (metastasis).

You may also have chemotherapy before surgery. It aims to shrink the cancer before surgery, making it easier to remove.

Targeted and experimental therapies

Researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”.

Targeted therapy is a treatment that stops cancer cells from growing and spreading. It targets specific characteristics within the cells.

Immunotherapy is a treatment that helps your immune system kill cancer cells.

Clinical trials have found that some targeted and immunotherapies work well for desmoplastic small round cell tumour. But, more research and trials are needed before these treatments are approved.

You might have a chance to join a study that looks into new ways to diagnose, treat, or use medications. Some studies also look at the care and well-being of patients. Your doctor or nurse can tell you about opportunities to join a clinical trial.

There are trials currently recruiting patients with desmoplastic small round cell tumour. For more information, please follow this link.

After treatment

After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-ups will usually include:

  • A chance to discuss symptoms.
  • An examination to look for any signs of the cancer returning. This may include scans such as CT or MRI scans.
  • A chest x-ray to rule out any secondary cancers occurring in the lungs.

Living with

After treating a previous tumour, desmoplastic small round cell tumour can come back in the same place. This is known as a “local recurrence.”

If the cancer reappears, you should have treatment as quickly as possible. Your treatment plan may include more surgery or radiotherapy, depending on what you need. You can check for recurrences by examining yourself. Your doctor or sarcoma nurse can tell you what to look for.

If the possibility of your cancer returning is a concern, please reach out to your doctor or nurse. They might move your follow-up appointment date earlier to look into your concerns.


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

A recurrence of desmoplastic small round cell tumour may be accompanied by cancer in other parts of the body. This is called “metastasis” or “secondary cancer”. Some people are diagnosed with sarcoma because their tumour has spread before the main tumour is found. Some patients may develop secondary cancers in the lungs. So you can check for this, a chest x-ray is done during follow-up appointments.

Secondary cancers may also appear in the liver and bones. If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

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 DSRCT.

Last reviewed: 19 October 2023

Next review due: 19 October 2026


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