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

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.

 

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.

 

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:

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.

 

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:

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.

 

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:

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:

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.

 

We have based this summary on information from the research team. The information they sent us has been reviewed by independent specialists (peer reviewed ) 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.