Treatment

In this section you will find information about the treatment methods (or modalities) for Ewing’s Sarcoma. We have tried to answer some of the key questions, but it is important to note that treatment decisions should be individualised for each patient. This should be the result of a discussion between all the professionals involved in a patient’s care.

This content is currently being updated and as such, is subject to pending updates. Please bear this in mind when reading this page at this time (31 March, 2016).

What are the common treatments used to treat Ewing’s Sarcoma?

What drugs (chemotherapy) may be used?

What might surgery involve?

When is radiotherapy used for treating Ewing’s Sarcoma?

What does each treatment do and why are they used?

 Introduction

Ewing’s Sarcoma has long been a subject of research, and as a result patient survival has improved dramatically over the past thirty years.

Research continues aiming to optimise and improve available treatments, reduce the risk of recurrence or spread of Ewing’s Sarcoma, and deliver new therapies tailored to the individual patient.

Ideally, patients should be treated within the context of clinical trials, which are generally now performed in collaboration between groups across many countries to improve the effectiveness of this research.

Patients will be treated under the guidance of a “Multi-Disciplinary Team” (MDT), which is the accepted method of delivering modern cancer care. This is a network of specialists that guide the patient through treatment options. To find out more about MDTs, click here.

The role of the MDT is to form a treatment plan incorporating all aspects of care with the patient and see that the best care is provided by professionals with specialist knowledge.

There is now a “National Ewing’s MDT” in England, which means all patients with a Ewing’s Sarcoma arising in the bones should be discussed by a panel of experts to help form a plan for their treatment, particularly surgical.

What are the common treatments used to treat Ewing’s Sarcoma?

There are several types of treatment that can be used to treat Ewing’s Sarcoma. Most commonly a combination of these methods (or multi-modality treatment) is used.

In general, chemotherapy is used in addition to local treatment of the primary tumour.

Treatment of the primary tumour is usually either surgical removal of the tumour, or radiotherapy, or a combination.

Chemotherapy

What is it?

This is use of drugs to treat cancer. Patients with Ewing’s Sarcoma receive a number of drugs with different properties to kill tumour cells.

What drugs are used?

Multi-drug treatment usually includes the following agents: Vincristine; Ifosfamide; Doxorubicin; Etoposide.

These four drugs form the “VIDE” chemotherapy protocol, which is the standard initial treatment for patients with Ewing’s Sarcoma in the UK. Patients generally receive six cycles of this before having local therapy to the primary tumour.

This may be surgery, and/ or radiotherapy.

Diagram of a PICC line, obtained from Macmillan

Diagram of a PICC line, obtained from Macmillan

After patients have completed VIDE chemotherapy, they generally have up to eight further cycles of “VAI” or “VAC” (abbreviations based on the drug names).

VAI uses ActinomycinD with Vincristine and Ifosfamide.

VIC uses Vincristine, Cyclophosphamide and Ifosfamide.

If a patient suffers from a relapse, where their Ewing’s Sarcoma returns, or the cancer spreads, there are a number of other drugs that can be used. These include high dose Ifosfamide, Cyclophosphamide/ Topotecan, Irinotecan/ Temozolomide and Gemcitabine/ Docetaxel.

High dose chemotherapy with stem cell rescue can also be used in some circumstances, using busulfan and melphalan.

Ifosfamide and Cyclophosphamide are given with Mesna, which protects the bladder from some of the side effects of these drugs.

Each case of Ewing’s Sarcoma is different.

This means that discussion and agreement between the specialists in the MDT are essential in finding the best therapeutic plan and providing the most effective treatment for each patient.

Ideally, patients should be entered into clinical trials where possible.

The current Ewing’s Sarcoma trial is called E.U.R.O. Ewing99. For more information on this trial, click here.

In the USA, the standard therapy uses the same drugs, but is given in a different schedule, called “VDC-IE”. For more information, click here.

The forthcoming EuroEwing2012 clinical trial will compare the effects of this regimen starting in late 2013 or early 2014, as E.U.R.O Ewing99 ended in September 2013.

Certain features of Ewing’s Sarcoma may influence what may happen to patients in the longer term.

Treatment may be adapted to take that into account.

How is chemotherapy given?

Chemotherapy is generally administered in a hospital. There are different methods of introducing drugs into the body, most commonly intravenously, through a Peripherally Inserted Central Catheter (PICC line), or Central Venous Catheter (central line). Central Venous Catheters are sometimes referred to as “Hickman” lines.

These are tubes placed into a blood vessel to allow the drugs to be injected directly over an extended period of time.

What are the side effects?

Chemotherapy works by targeting cells that are dividing quickly. Unfortunately these drugs do not only affect cancerous cells, and can cause a number of side effects.

The most common side effects include sickness, hair loss, tiredness, anaemia, risk of infection, bleeding and bruising. This is because the drugs stop the bone marrow producing red blood cells, white blood cells and platelets (which help blood clot).glossary

There are also more specific side effects for each drug, and the drug can affect organs such as the heart and kidneys. If you would like to know more about the side effects of chemotherapy, click here.

Surgery

Why is surgery used?

Surgery has two general aims:

  • Complete removal of the tumour 
  • Preservation of the limb without loss of function, if the latter is possible.

It is important to leave a wide margin of normal tissue around the tumour to make sure that all the tumour cells are removed as this will reduce the risk of recurrence (where the tumour comes back in the same place).

If surgery can only remove part of the tumour it should be avoided.

What goes into the decision?

Planning before the operation is required. MDT members will review the case, guided by the needs of the patient.

Magnetic-Resonance Imaging (MRI) will guide the radiologists and surgeons as to the site and type of surgery.

A general anaesthetic is normally required. The anaesthetist will see the patient before the operation to explain the risks and ensure the patient understands the procedure.

The anaesthetist will also discuss pain relief to be used, the possibility of an epidural, and patient controlled pain-relief after the procedure.

What are the different options?

Endoprostheses, removal, irradiation and re-implantation of the bone or occasionally allografts are all surgical options when trying to salvage a limb. In some cases limb salvage may not be possible and an amputation may be necessary.

There are two types of prosthesis:

Diagram of an endoprosthesis, obtained from Cancer Research UK

Diagram of an endoprosthesis, obtained from Cancer Research UK

  • An endoprosthesis is a prosthetic entirely within the body. It normally comprises a joint replacement of variable length that replaces the diseased bone which has been removed and is then attached to the remaining host bone by a stem.

Improvements in endoprostheses mean that the prosthesis can be lengthened as the child grows to maintain equal limb-length. Modern advancements mean this lengthening can be done in the outpatient clinic.

  • The other type of prosthesis is external, and replaces a limb that has been amputated. Improvements in prosthetic technology have been considerable in recent years, greatly increasing function post-operation.

Other surgical options may include:

  • Re-implantation is where the tumour is removed, and treated using either radiotherapy or another form of sterilisation, before being re-implanted into the original location. 
  • Allografts are transplants of tissue from another part of the body, or a donor, to replace tissue that has been removed. Allografts are rarely used in the UK. 
  • Rotationplasty involves removing the joint below the one removed and reattaching this. For example, an ankle can be removed, rotated and attached to form a knee joint, allowing near-normal knee function. These operations are rarely performed in the UK.

Amputation may be the only option to safely remove the tumour. The MDT will discuss with the patient if this is possible prior to the operation to provide as much information as possible.

This is to allow the patient to make an informed decision about their care. As mentioned above, all patients treated in England should be discussed at the National Ewing’s MDT.

In the event of recurrence, the MDT may involve other specialist surgeons to assess and operate on the patient. The surgeon consulted will depend on the site of the tumour or metastases.

Radiotherapy

What is it?

Radiotherapy uses high-energy rays to destroy cancer cells, whilst doing as little damage as possible to surrounding cells.

The final component in the multi-modality approach is radiotherapy. It may not be appropriate in all cases of Ewing’s Sarcoma, but is important for many patients.

Why is it used?

After being diagnosed, radiotherapy can be a useful tool in reducing tumour size in patients who have a large tumour causing pain. In this instance, surgery would cause a loss of function.

By reducing the volume of the tumour, the surgeon has a better chance of performing a successful operation and unwanted symptoms can be reduced or resolved completely.

This is called neo-adjuvant or pre-operative radiotherapy.

After surgery, radiotherapy may be used to improve control of the tumour site and help minimise risk of recurrence. This is particularly important where there are still live tumour cells in the resection specimen, or in some patients who were deemed to be high risk.

Improvements in radiotherapy techniques have led to reduced long-term effects. Risks still exist, but the potential benefits outweigh these dangers.

Radiotherapy can also be used to help control symptoms related to tumours or metastases. This is generally referred to as palliative therapy.

How is it decided whether to have radiotherapy?

As with other aspects of treatment, the MDT will decide the best treatment for the patient and offer as much information and support as possible.

For more information on radiotherapy and its side effects, click here.

Once treatment is complete, regular follow-up by oncologists and surgeons will be necessary to assess the outcome of the treatment and monitor for recurrence or spread. It will also monitor for long-term side effects of treatment, and enable access to other appropriate services such as physiotherapy.

The future

Survival rates have improved since the use of chemotherapy began, but they have reached a plateau recently, meaning new treatments are required.

Research is ongoing to try and improve the outcome for patients with Ewing’s Sarcoma.

As we learn more about the biology of Ewing’s Sarcoma, novel treatments will emerge.

Agents that specifically target the tumour are very attractive to researchers. The EWS-ETS transcription factors, which aid the initiation of the tumour, may be an exciting area of research.

Optimising existing treatments such as the quality of prostheses may also help minimise the impact on quality of life for these patients.

Production date: March 2013

Review date: Currently under review

ESRT commits to producing high-quality, reliable medical information. For more information about the sources used in the production of this material, please contact ewingssarcoma.researchtrust@gmail.com. We would also welcome feedback on the information that we produce, so if you wish to let us have your views please send your comments to the charity at the same email address.