A trial looking at the treatment of children and young people with non rhabdomyosarcoma soft tissue sarcomas (EpSSG NRSTS 2005, STS 2006 03)

Cancer type:

Children's cancers
Sarcoma
Soft tissue sarcoma

Status:

Results

Phase:

Phase 3

This trial was done to find out more about the best way to treat children and young people with a group of soft tissue sarcomas called non rhabdomyosarcomas.

More about this trial

Soft tissue sarcomas are cancers of the supporting tissues of the body such as muscle, nerve or fat. There are several different types of sarcoma.
 
Children with soft tissue sarcoma are usually put into one of two groups:
  • rhabdomyosarcoma
  • non rhabdomyosarcoma - including synovial sarcoma, fibrosarcoma, alveolar soft part sarcoma and rhabdoid tumours
When this trial was done, doctors usually treated children with non rhabdomyosarcoma with surgery. And sometimes chemotherapy or radiotherapy, or both.
 
In this trial, doctors treated children with non rhabdomyosarcoma according the same treatment plan (protocol). This protocol was called EpSSG. The treatment varied depending on the type of sarcoma.
 
Doctors had used the results of earlier trials to decide which treatments to include in the EpSSG treatment protocol. 
 
The aim of this trial was to collect more information about how well treatment for non rhabdomyosarcoma works. And to use this information to improve treatments in the future.

Summary of results

The research team looked at the treatment for several different types of sarcoma separately. There is information about each one below.
 
This trial was open for children to join between 2005 and 2016. The research team first published results in 2015.
 
Results for synovial sarcoma
Synovial sarcoma is a cancer that develops around joints and tendons, often around the knee.
 
A total of 138 children and young people joined this part of the trial. 
 
The treatment they had depended on whether their sarcoma was more likely to grow and spread (high risk) or less likely to grow and spread (low risk).
 
The research team looked at how many children were living 5 years after treatment. They found that overall it was more than 9 out of 10 (91%). This is higher than in some other clinical trials.
 
Those with low risk synovial sarcoma had surgery but didn’t have chemotherapy. The results showed that all 24 children in this group were living 3 years after treatment.
 
Those with medium (intermediate) risk synovial sarcoma had ifosfamide and doxorubicin chemotherapy after surgery. Some also had radiotherapy. The results showed that all 37 children in this group were living 3 years after treatment.
 
Those with high risk synovial sarcoma had chemotherapy before their operation (neoadjuvant chemotherapy). Some also had radiotherapy afterwards. The results showed that 128 out of 138 children (92%) in this group were living 3 years after treatment.
 
The research team concluded that not everyone with synovial sarcoma needed chemotherapy. The trial was too small to say for sure if children needed radiotherapy or not. But the trial team think it may be ok for some children not to have it. 
 
They suggest other trials are done to find out more about the best combinations of treatment for these patients.
 
Results for infantile fibrosarcoma
Infantile fibrosarcoma (IFS) is a rare cancer that starts in the connective (fibrous) tissue of very young children. It often grows quickly but doesn’t usually spread to other parts of the body (metastasise).
 
A total of 50 children from various countries joined this part of the trial. 
 
They were all under 2 years old and due to have surgery to remove their fibrosarcoma. They were grouped depending on how the operation went:
  • 11 were IRS-1 which means the surgeons removed all the sarcoma
  • 8 were IRS-2 which means the surgeons removed all the sarcoma they could see, but there may be other cancer cells there
  • 31 were IRS-3 which means they surgeons couldn’t remove all of the sarcoma
Those that were in the IRS-1 group didn’t have any more treatment after surgery. 
 
One child in the IRS-2 group had chemotherapy, but the rest didn’t need to. 
 
In the IRS-3 group, 27 had chemotherapy after surgery. Most had a combination of vincristine and actinomycin (VA). After that, 1 had radiotherapy and 19 had another operation.
 
When the trial team looked at how well all the treatment had worked, they found that the sarcoma had gone away completely in 42 out of 50 children (84%). And that 3 years after treatment, 47 out of 50 (94%) were living.
 
They concluded that it is possible for children with IFS to do very well without needing too much treatment. And that the combination of vincristine and actinomycin was the best chemotherapy to use.
 
They also highlighted how important it is for research teams around the world to work together to find the best treatments for rare cancers.
 
Results for alveolar soft part sarcoma
Alveolar soft part sarcoma (ASPS) is a rare form of sarcoma which often starts in the arms or legs.
 
A total of 22 children aged 2 to 17 years old joined this part of the trial. 
 
The treatment they had depended their individual situation. Doctors looked at factors such as how big the sarcoma was, and whether it had spread to another part of the body.
 
Of the 22 who took part:
  • 14 had surgery but didn’t need any other treatment
  • 4 had surgery and then radiotherapy
  • 1 had surgery and then chemotherapy and radiotherapy
  • 1 had chemotherapy before surgery, and then radiotherapy
  • 2 had chemotherapy alone
The trial team looked at how well the various treatment options worked. The results showed that all the children taking part were living 5 years after treatment.
 
They concluded that it was possible to run a trial for ASPS, and that treatments work well.
 
Results for rhabdoid tumours
Extracranial malignant rhabdoid tumours (EMRT) are rare and often grow quite fast.
 
A total of 100 children, aged 3 to 10 years old, from 12 countries joined this part of the trial. Of the 100 who took part:
  • 77 had sarcoma in one part of the body
  • 23 had sarcoma that had spread to another part of the body
They were all due to have surgery followed by chemotherapy and radiotherapy. The chemotherapy drugs included vincristine, cyclophosphamide, doxorubicin, carboplatin and etoposide.
 
Of the 100 who took part, 43 had all the treatment as planned. Some stopped treatment early because their sarcoma started to grow or because of the side effects they were having. Or because the doctor decided it was the best option for them.
 
The trial team looked at how well the treatments worked and found that 38 out of 100 children (38%) were living 3 years after diagnosis. 
 
They then compared results for children whose cancer had spread and those whose cancer was localised in one area. They found that:
  • 31 out of 77 children (40%) with localised sarcoma were living 4 years after diagnosis
  • 3 out of 23 children (13%) with sarcoma that had spread were living 2 years after diagnosis
The research team concluded that this combination of treatment may work for some patients with EMRT, but that it’s a difficult sarcoma to treat.
 
Where this information comes from
We have based this summary on information from the research team. The information they sent us has been reviewed by independent specialists (peer reviewed Open a glossary item) and published in medical journals. The figures we quote above were provided by the trial team who did the research. We have not analysed the data ourselves.

Recruitment start:

Recruitment end:

How to join a clinical trial

Please note: In order to join a trial you will need to discuss it with your doctor, unless otherwise specified.

Please note - unless we state otherwise in the summary, you need to talk to your doctor about joining a trial.

Chief Investigator

Dr B. Brennan

Supported by

Central Manchester University Hospitals NHS Foundation Trust
Children's Cancer and Leukaemia Group (CCLG)
NIHR Clinical Research Network: Cancer

Freephone 0808 800 4040

Last review date

CRUK internal database number:

768

Please note - unless we state otherwise in the summary, you need to talk to your doctor about joining a trial.

Around 1 in 5 people take part in clinical trials

3 phases of trials

Around 1 in 5 people diagnosed with cancer in the UK take part in a clinical trial.

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