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Treatment of Ewing’s sarcoma
Patients with Ewing’s sarcoma have different treatment plans depending on the location and stage of the disease. Typically, treatment for Ewing’s sarcoma involves killing cancer cells throughout the body with chemotherapy, then treatment of the primary tumour with surgery and/or radiotherapy. Chemotherapy is usually given again after these initial treatments to try and make sure there are no cancer cells left anywhere in the body.
A multidisciplinary team (MDT) will manage a patient. This is a group of healthcare professionals who are experts in their various fields and they work together to ensure a patient is properly diagnosed and if necessary, treated.
The National Ewing’s sarcoma Multidisciplinary Team Meeting was formed in 2011 and is a unique national forum set up to improve the treatment planning and choices made for Ewing’s sarcoma patients. This forum brings together clinicians and specialists from around the UK to discuss the treatment management and plans for current patients.
During a patient’s treatment, they will also be looked after by other health professionals including nurse specialists, physiotherapists to help with rehabilitation after surgery, dieticians to ensure proper nutrition and social workers/psychologists to help with emotional, social and educational needs.
Types of Treatment
Chemotherapy given before surgery is called “neoadjuvant chemotherapy”. Patients are given a combination of chemotherapy drugs. In Ewing’s sarcoma, drugs are usually delivered as an injection or infusion directly into the blood. The aim of treatment at this stage is to shrink the tumour and kill any cancer cells that may have already spread.
Although the exact treatment plan and chemotherapy drugs used may vary as part of multinational trials, such as Euro Ewing 2012, the most common treatment for Ewing’s sarcoma before surgery is the combined use of four chemotherapy drugs given together. These drugs are vincristine, ifosfamide, doxorubicin and etoposide. This combination of drugs is called “VIDE”. The purpose of using four different drugs is to maximise their effect. If the cancer stops responding to one of the drugs then the aim is that the other drugs will still be effective in killing the cancer cells.
Each course of VIDE is given over 3-4 days every 3 weeks (1 cycle) for 6 cycles. This takes about 18 weeks in total. Response to treatment is regularly assessed by MRI scans throughout this period and the patient will be seen frequently by their medical team to assess chemotherapy side effects and toxicity.
Each patient responds differently to chemotherapy. Some patients may have a very mild reaction, other patients may react strongly. Typical symptoms can include:
- Nausea (feeling sick)
- Loss of appetite
- Hair loss
- Dry mouth and ulcers
There is research to show that light exercise such as walking and swimming can help alleviate some of the symptoms.
Radiotherapy is designed to treat the tumour and not the whole body. Radiotherapy involves using high energy particles or waves, such as X-rays and gamma waves, to kill the cancer cells that make up the tumour. The high energy beams damage the DNA inside cancer cells to prevent the cells from dividing and cause them to die. Radiotherapy is usually given as a single dose each day (a few minutes) for 5-6 weeks. The symptoms of radiotherapy are similar to chemotherapy but may also include sore skin where the energy beams are focused. Radiotherapy can be given during chemotherapy cycles, before and after surgery.
Proton Beam Therapy
As of 2018, a new type of radiotherapy called proton beam therapy will be offered to certain patients in the UK. This type of radiotherapy uses proton beams instead of x-ray beams. Although the radiotherapy dose to the tumour is essentially the same, protons cause less damage to the tissues they pass through. This means that doctors can administer a high dose of radiotherapy, with less effect on normal healthy tissue. Two proton beam therapy centres are being developed, one at the Christie Hospital in Manchester and one at UCLH in London. These are due to start treating patients in 2018 and 2020 respectively.
There have been very few studies looking at the effectiveness of proton beam therapy in bone cancer patients. One study in America looked at its effectiveness in osteosarcoma and showed that it was effective in some patients. Once the UK is treating patients, more studies will be needed to fully assess the effectiveness of this treatment in bone cancers such as Ewing’s sarcoma.
The multidisciplinary team (MDT) at the Bone Tumour Centre makes the decision whether surgery is possible. There are two aims of surgery:
1. to remove the tumour from the body,
2. to reconstruct the resulting bone defect.
Surgeons have developed different surgical techniques in reconstructing the bone after removal of the Ewing’s sarcoma in order to preserve the function of the limb. The most commonly used reconstructive technique is replacement of the affected bone with a metal implant (prosthesis).
Other techniques for reconstruction include:
1. a bone transfer (usually the fibula bone from the lower leg or part of the pelvic bone),
2. allografts (where donated bone from a bone bank is used to rebuild the limb), and
3. irradiation/re-implantation which involves removing the bone containing the tumour and treating with a very high dose of radiotherapy; the bone can then be put back into the patient).
Despite advances in surgical techniques around 10% of patients require an amputation (removal of the limb) to safely remove the tumour. A prosthetic (artificial) limb can be made for the patient in these cases.
All types of surgery to remove tumours can be very complicated and require careful individual planning for each patient, including patients undergoing a planned surgical resection (i.e. removal of all or part of tissue, structure or organ). All patients undergoing planned surgical resection of Ewing’s sarcoma are discussed before surgery at the National Ewing’s sarcoma Multidisciplinary Team Meeting.
“Adjuvant chemotherapy” is chemotherapy that is given after surgery. The aim is to clear up any remaining cancer cells that have survived the previous treatments. The number of chemotherapy drugs and length of treatment will vary and again is usually as part of a multinational trial. The most common chemotherapy regime after surgery for Ewing’s sarcoma is a combination of three to four drugs – vincristine, actinomycin D plusifosfamide or cyclophosphamide. Adjuvant chemotherapy usually lasts for 24 weeks after surgery.
Produced: March 2018
Published: December 2018
Review: December 2021
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