A trial looking at treatment for patients with Ewing's Sarcoma or peripheral Primitive Neuroectodermal Tumour (pPNET) (EURO-EWING 99)

Please note - this trial is no longer recruiting patients. We hope to add results when they are available.

Cancer type:

Children's cancers
Ewing's sarcoma




Phase 3

This trial is looking at chemotherapy, surgery and radiotherapy for children, young people and adults with Ewing’s sarcoma or primitive neuroectodermal tumour (PNET). This trial is supported by Cancer Research UK.

This trial is for children and adults up to the age of 50. We use the term ‘you’, but of course if you are a parent, we are referring to your child.

Ewing's sarcoma and peripheral Primitive Neuroectodermal Tumour (PNET) are similar types of cancer mostly affecting teenagers, young adults and children. These cancers mainly develop in or near the bones.

Ewing's sarcoma and PNET are usually treated with chemotherapy first. This is usually followed by surgery, radiotherapy and further chemotherapy, or a combination of these. This trial will compare several different combinations of chemotherapy drugs to see if they are more effective or have fewer side effects than the standard treatments normally used.

Who can enter

This trial is open to children and adults up to the age of 50 years.

You can enter this trial if you

  • Have been diagnosed with Ewing’s sarcoma or PNET
  • Have satisfactory blood results

You cannot enter this trial if you

  • Have any other medical condition or illness that means you cannot have the treatment given in this trial
  • There is a delay of more than 45 days between a tissue sample being taken from the cancer and registration on to the trial or the start of chemotherapy
  • Are over 50 years of age

Trial design

This is a phase 3 trial. if you take part you will have a central or long line put in. It makes it easier to give chemotherapy and take blood samples.

First, you will have a course of chemotherapy called ‘induction’ chemotherapy. The drugs are called vincristine, ifosfamide, doxorubicin and etoposide. This is usually given the shortened name of VIDE. These drugs are given through the central line over 3 days in 3-week cycles.

This treatment takes at least 18 weeks. Your treatment may take longer if your chemotherapy is delayed by side effects (see below). During this time you will have stem cells collected. Stem cells are very early blood cells, which are collected and stored in case you need them in the future to replace blood cells damaged by chemotherapy. This is called a stem cell transplant Open a glossary item.

After the induction chemotherapy, you may have surgery to remove the cancer. Your oncologist, surgeon and radiotherapy specialist will decide what further treatment you should have and this will be discussed with you.

You will then be put into 1 of 4 treatment groups, depending on

  • The size of the tumour
  • How well the chemotherapy has worked
  • Whether the cancer has spread or not and where it has spread to

Everybody will have one cycle of treatment with VAI (vincristine, actinomycin D and ifosfamide). Chemotherapy after that is different for each group.

If you are in group 1, the treatment given so far has worked well and your cancer has not spread. You will have 7 more cycles of VAI.

People in groups 2 and 3 will be randomised to have one of two different treatments. Neither you nor your doctor will be able to decide which treatment you have.

If you are in group 2, the treatment given so far has not worked as well and the doctors were not able to remove all of the cancer during your operation. You will have either

  • 7 more cycles of VAI or
  • 1 cycle of busulphan and melphalan chemotherapy followed by a stem cell transplant Open a glossary item

If you are in group 3, your cancer has spread to your lungs. You will have either

  • 7 cycles of VAI (vincristine, actinomycin D and ifosfamide) and radiotherapy treatment to the lungs or
  • 1 cycle of high dose busulphan and melphalan chemotherapy, followed by a stem cell transplant Open a glossary item and no radiotherapy

If you are having a stem cell transplant, you will have it the day after chemotherapy has finished. This means the stem cells collected from you at the beginning of treatment will be given back through a drip into your central line.

If you are in group 4, your cancer has spread to places other than the lungs. The commonest sites for spread are other bones and, or the bone marrow. After the initial chemotherapy, your treatment will be decided on an individual basis. You may be offered treatment as part of another trial.

Hospital visits

Before your treatment, you will have a biopsy of your cancer. The researchers may also ask your permission to keep samples of your cancer to use in future research into the causes and behaviour of this type of cancer. Before, during and after your treatment, you will have a number of tests. These include

The treatment given for Ewings and PNET is very intensive. Your will be in hospital on and off for some months. This will vary depending on the type of treatment you have.

When the treatment has finished, a doctor will see you

  • Every month for 6 months, then
  • Every 2 months for 6 months
  • Every 3 months for a year
  • Every 6 months for 3 years
  • Every year after that

Side effects

All chemotherapy drugs have side effects. The most common side effects are

There is more information about the particular side effects of ifosfamide, vincristine, doxorubicin, etoposide, actinomycin D, cyclophosphamide, busulphan and melphalan in the main cancer drugs section of CancerHelp UK.

There is information about the side effects of radiotherapy on CancerHelp UK. The most common side effects of radiotherapy to the lungs are

  • Sore or dry skin
  • Tiredness
  • Sore throat or trouble swallowing
  • Feeling sick

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 Bernadette Brennan

Supported by

Cancer Research UK
Cancer Research UK Children's Cancer Trials Team
University of Birmingham
European Organisation for Research and Treatment of Cancer (EORTC)
Experimental Cancer Medicine Centre (ECMC)
NIHR Clinical Research Network: Cancer

Other information

This is Cancer Research UK trial number CRUK/02/014.

Questions about cancer? Contact our information nurses

Freephone 0808 800 4040

Last review date

CRUK internal database number:

Oracle 278

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.

Last reviewed:

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