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What is Ewing Sarcoma?
(family of tumours, bone and soft tissue cancer)

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Introduction / About this FAQ

The contents of this document have been prepared by an international panel of experts to answer some of the frequently asked questions (FAQ) about bone cancer. The project has been supported by SIOP (International Society of Paediatric Oncology) Scientific Committee.

As the editorial panel answer new questions these will be added see the reference at the bottom of this page.

Please note:

  • The main focus of the FAQ is on cancer that has started in the bone (primary bone cancer) rather than cancer that started in another part of the body and later spread to the bone.
  • The FAQ is intended to provide a supplementary source of information about bone cancers. If you are a patient or parent of a child with bone cancer your doctor should be able to provide you with the information which is most relevant to you.
  • It is not designed to provide medical advice and cannot be a substitute for professional medical services.
  • The FAQ has a section on general questions about bone cancer and separate pages about Ewing's sarcoma and Osteosarcoma (see the reference at the bottom of this page).

Editorial Panel

  • Dr G Bacci
  • Prof. S Burdach
  • SJ Cotterill
  • Prof. AW Craft
  • Mr R Grimer
  • Prof H Jurgens
  • Prof R Kotz
  • Dr H Kovar
  • Dr PA Meyers
  • Dr O Oberlin
  • Dr P Picci
  • Dr G Saeter
  • Dr D Spooner

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What is Ewing's sarcoma / PNET?

Ewing's sarcoma is a cancer. The cancer can start in bone or in soft tissues. The most common sites for Ewing's sarcoma are the pelvis, the thigh, and the trunk of the body. The peak ages are between 10 and 20, but younger children and older adults can also get Ewing's sarcoma. We do not know exactly what kind of cell gives rise to Ewing's sarcoma. It has some features that resemble the early cells that would normally develop into part of the nervous system. We do not know what causes Ewing's sarcoma.

The most common early signs of Ewing's sarcoma are pain and swelling. Like other sarcomas, Ewing's sarcoma can spread to other parts of the body. Even when the tumour is detected at a very small size, there may be evidence of microscopic spread. For this reason, Ewing's sarcoma always requires treatment to the whole body. This treatment includes chemotherapy. Chemotherapy is intended to destroy the tumour cells which have spread to the rest of the body and to shrink the main mass of tumour cells. Successful treatment also requires another form of treatment to the main mass of tumour. This can be surgery, radiation therapy, or a combination of the two.

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What are the symptoms of Ewing's sarcoma?

Symptoms of Ewing's sarcoma vary from person to person and depending on the location and size of the cancer. The most common symptoms are pain and swelling or tenderness in the affected area. Pain may become very intense when the tumour is located near important nerves, like in the sacrum, pelvis or spine. Swelling is often seen, especially when the log bones of the arms or legs are affected.

Sometimes the tumour can interfere with movement and can weaken the bones, occasionally leading to a fracture. Other symptoms of cancer may include tiredness, fever, weight loss, and anaemia. None of these symptoms is a sure sign of cancer; if you suspect you have a health problem consult your doctor.

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How is Ewing's sarcoma diagnosed?

If a bone tumour is suspected the doctor will do a complete medical examination. This may include a blood test as bone tumours can be associated with increased levels of certain enzymes in the blood. The doctor may also recommend X-rays and other scans of the bone(s), if X-rays and scans suggest that a tumour might be present then a biopsy (removal of a sample of tissue) will be performed. A pathologist will then examine the cells to determine whether it is cancerous, and if so what type of cancer it is. Ewing's sarcoma is composed of small-blue-round cells, these can appear similar to other types of cancer so special stains and other laboratory tests are needed to make the diagnosis.

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What is the treatment for Ewing's sarcoma?

Ewing's sarcoma is usually sensitive to chemotherapy and radiotherapy. Modern treatments are based on chemotherapy combined with local therapy (surgery and/or radiotherapy to the main tumour):

  • Chemotherapy (using drugs to kill cancer cells)
  • Surgery (to take out the tumour in an operation)
  • Radiotherapy (using high-dose x-rays to kill cancer cells)

Chemotherapy is given to kill malignant cells that may be circulating around the body. It is generally administered before and after the local therapy. The choice of local treatment (surgery and/or radiotherapy) will depend on the size and location of the tumour, if the cancer has spread or not, and other individual factors. Due to progress in limb-salvage surgery and awareness of problems associated with radiotherapy, surgery is the most frequent type of local therapy. Radiotherapy is usually reserved for tumours that are difficult to reach surgically or locations associated with surgical complications (e.g. spine, pelvis and skull). Sometimes radiotherapy is given as well as surgery, particularly following marginal resections.

Treatment of bone cancers is complex and involves a team of different specialists usually within an institution that is experienced in treating these types of cancers.

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What is the chance of recovery?

Overall, the chance of recovery (prognosis) for Ewing's sarcoma / pPNET has dramatically improved since the development of modern chemotherapy.

The chance of recovery will depend on a variety of influences; if the cancer has spread, the size of the tumour, location, the person's general health and other individual factors. Also important is how the cancer responds to treatment; how much of the main tumour can be removed/destroyed by surgery and/or radiotherapy, and the number of cancer cells killed by chemotherapy.

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Which bones can be affected by Ewing's sarcoma?

The most frequent locations for the primary tumour are the pelvis, femur (thigh bone), tibia / fibula (bones of the lower leg), bones of the spine, ribs and humerus (upper arm). Other sites are less common, however, Ewing's sarcoma can potentially arise in any of the 206 bones in the body. It can also develop in the soft tissues without bone involvement.

The figure below gives a summary of primary tumour site in a series of over 900 people diagnosed with Ewing's sarcoma of bone.

Image - Which bones can be affected by Ewing's sarcoma?

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Is Ewing's sarcoma a childhood or an adult disease?

Approximately half of all people with Ewing's sarcoma of bone are under 15 years of age at diagnosis. However, it is also common in young adults. The peak ages are between 10 and 20. It is less common before the age of 5 and after the age of 30. Sometimes young adults may be treated by a "paediatric" oncologist because of the doctor's experience with treating this type of cancer.

Images - Is Ewing's sarcoma a childhood or an adult disease?

The figure above shows the age distribution for over 900 people with Ewing's sarcoma of bone registered with clinical trial groups in Germany and the UK.

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Are children given the same treatment as adults?

Ewing's sarcoma or PNET is a type of cancer found in children and young adults, with a peak incidence of between ages 10 and 20. It is less common in children under 5 or in adults over 30. There is no rationale for treating children and adults differently; chemotherapy is usually the same. However, children are thought to tolerate chemotherapy better than adults. Also children can develop more severe delayed late effects from radiation therapy, such as bone growth retardation. This is one of the factors taken into account to decide the type of local therapy given.

Several studies have shown similar results in adults and children when they are treated with the same protocol.

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What is the cause of Ewing's sarcoma?

The cause of Ewing's sarcoma / pPNET remains unknown.

In young people, the development of the tumour appears to be in some way related to periods in life with rapid growth, hence the average for tumour development is 14-15 years. The relationship to growth may also be part of the explanation why Ewing's sarcoma is slightly more common in boys than in girls. However, Ewing's sarcoma remains an extremely rare tumour in all groups of the population, and there is no extra cause for concern in rapidly growing teenagers. The relationship between bone growth and Ewing's sarcoma is thought to be due to an increased vulnerability of rapidly growing cells to damage caused by chance or by as yet unidentified factors.

Sometimes the person with Ewing's sarcoma or the parents relate a previous injury or trauma to the development of the tumour. However, medical research has not found any proven relationship between such injury and the risk of subsequently developing Ewing's sarcoma.

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Are my relatives at risk?

Almost all Ewing's sarcoma tumours have a change in their genetic makeup. Normal human cells contain 23 pairs of chromosomes. In Ewing's sarcoma tumours, a piece of chromosome 11 has moved to chromosome 22. This creates a new piece of DNA. This does not mean that the disease is passed from parent to child. For genetic change to pass from parent to child, it must be present in the sperm or egg which give rise to a new human being. The genetic change in Ewing's sarcoma tumours occurs only in the tumour cells, not in the sperm or egg. Patients who get Ewing's sarcoma did not inherit the disease from their parents. Patients who survive Ewing's sarcoma and have children do not pass on an increased risk of cancer to their children. The brothers and sisters of patients with Ewing's sarcoma do not have an increased risk for Ewing's sarcoma.

There may be some influence of genetics in the chances of developing Ewing's sarcoma. Asians and American blacks have a much lower risk to develop Ewing's sarcoma than Caucasians.

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Is primitive neuroectodermal tumour (PNET) different to Ewing's sarcoma?

Peripheral primitive neuroectodermal tumours (PNET, or more correctly pPNET) start in bone or soft tissues. Like Ewing's sarcoma (ES) they are composed of small-blue-round cells. They differ from ES in that they show more developed features of cells associated with the nervous system.

Genetic analyses show that both pPNET and ES share a unique genetic alteration, an exchange of material between chromosomes 11 and 22. Also, ES and pPNET have a similar response to chemotherapy. Based on these and other similarities Ewing's sarcoma and pPNET are regarded as closely related members of the same family of tumours.

Treatment for pPNET is the same as that for Ewing's sarcoma. The answers in this FAQ apply to both Ewing's sarcoma and pPNET.

Note: brain tumours are also frequently referred to as primitive neuroectodermal tumours (PNET). These are very different to pPNET of bone; they do not share the same 11;22 translocation and require different treatments.

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What are Askin's tumours?

Askin's Tumours (AT) are malignant round cell tumours of the thoracopulmonary region (chest wall). Studies have shown cell characteristics of Askin's Tumours are similar to those of Ewing's sarcoma and primitive neuroectodermal tumours (PNET), and that a common genetic feature (11;22 chromosomal translocation ) is shared by these three entities. These findings suggest that Askin's Tumours, Ewing's sarcoma and PNET represent different manifestations of a single tumour type (the "Ewing's sarcoma family of tumours").

Clinically, Askins tumours present as a mass in the periostium and / or soft tissue of the chest wall. The treatment of this tumour is local therapy (surgery and / or radiotherapy) combined with systemic chemotherapy. The drugs used for chemotherapy are generally the same as those used to treat Ewing's sarcoma / PNET.

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How does the Ewing's sarcoma spread through the body?

Ewing's sarcoma spreads when tumour cells enter the blood supply and are circulated to other parts of the body where they may form secondary tumours ("metastases"). Chemotherapy is given to kill these circulating tumour cells. The most common sites for secondary tumours are the lungs and other bones. Tumour cells may also spread via the lymphatic system (this is a network of lymph glands around the body). Also, tumours can spread by direct growth of the primary tumour to form "skip metastases", though these are rare.

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Has the delay in diagnosis affected outcome?

Delays in diagnosis of Ewing's sarcoma are extremely common. The average duration of symptoms is 20 weeks and for patients with tumours of the pelvis it is not uncommon for patients to have had symptoms for one year or more.

The first symptoms experienced by most patients are rather non-specific pain which gradually becomes more severe and persistent. Many patients will have been investigated for a variety of conditions and some will have had treatment including operations which have not resolved the problem. Up to 25% of patients will have had an X-ray which has either not been of the right part of the bone or has failed to detect the abnormality in its early stages.

No-one has ever proven that an actual delay in diagnosis has directly affected prognosis for Ewing's sarcoma. This is somewhat surprising as tumour size has been shown to be associated with overall survival in that smaller tumours tend to have a better outlook. The link between tumour size and duration of symptoms is however not clear. some patients will present very late with small tumours, whilst others will have a very short history and yet have a very large tumour.

Whilst tumour size is an important prognostic factor the actual responsiveness to chemotherapy is probably more important and this is not necessarily related to tumour size in any way. Some tumours will always be sensitive to chemotherapy, whilst others will not.

The presence of metastatic disease is another poor prognostic factor and whilst metastases are more common in large tumours, there is no clear cut relationship that identifies when metastases will be released and become detectable.

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What are the long-term problems of endoprosthesis?

An endoprosthesis is an artificial bone replacement within the body, for example a metal rod replacing a bone, while leaving the surrounding tissues (muscle, skin, blood vessels, nerves etc) in place. Endoprostheses are often used in limb-sparing surgery for bone tumours. Some people have no problems with their endoprosthesis. However, there are potential long-term problems, these will vary from person to person.

Like any other prosthesis the tumour endoprosthesis can have problems with wear. In the case of a knee amputation there is usually a hinge necessary to compensate for muscle and ligament resection. The axis is under stress and may have to be revised after wear. Therefore, after 5 to 10 years a rebrushing of polyethylene parts can be necessary. In case of metal-metal axis there can be some metallosis, but duration of the material is much longer and may last 20 years or more.

Another problem can be late infections. This can require further surgery to revise the prosthesis, and in the majority of cases a one-step revision is possible.

Also, the long-term durability of stems and body of the prosthesis can be problematic. Cemented as well as non-cemented stems may break after some time depending on the activity, weight and height of the patient. Also, stress shielding problems can occur at the anchorage which in turn may cause fracture of the stem. Sometimes motion-associated soft tissue corrosion may occur. In children, a thick fibrous tissue may develop around the prosthesis possibly inhibiting motion after some years which requires revision and resection of the fibrous tissue sleeve.

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What is an 11;22 translocation?

Every cell of the body contains the genetic information needed to create a new person. This information is contained in millions of genes encoded on a large densely packed molecule, called DNA. At a specific stage of cell division the DNA becomes visible under a microscope as a set of 23 chromosomes, each pair consisting of one chromosome copy from the father and another from the mother. The chromosome pairs differ in length and structure. Based on their individual sizes the chromosomes are numbered from 1 to 22. The 23rd pair is the sex determining (X and Y) chromosomes.

In a cancer cell the genetic information may contain mistakes and rearrangements. Some of these defects can be seen under the microscope as altered chromosome structures. In the Ewing's sarcoma family of tumours a piece of chromosome 11 has moved to chromosome 22 and a piece of chromosome 22 has moved to chromosome 11. The result of this so called translocation is a recombination of two unrelated genes to form new genetic information This novel gene appears to be involved in the abnormal regulation of other genes. The 11;22 translocation and resulting "fusion gene" are specifically found in Ewing's sarcoma and closely related tumours and can therefore be used to help in the diagnosis of this disease.

Information Reference:

The information contained within this page was obtained from the Children's Cancer Web (Cancer Index)

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