:: Ewings Sarcoma Facts ::

Who was Ewing's sarcoma named after ?

James Ewing, 1866-1943, first described the tumour that was to be named after him in the 1920's. It was Ewing's work which established that the disease was separate from lymphoma and other types of cancer know at that time. Ewing was originally born in Pittsburgh and embarked on his medical career in 1888. In 1899 he was appointed as the first Professor of Pathology at Cornell University where he developed a keen interest in cancer. He was a co-founder of both the American Association for Cancer Research in 1907 and the American Cancer Society in 1913. He was also a pioneer in the use of radiotherapy in the treatment of cancer.

James Ewing died from bladder cancer at the age of 76.

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.

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.

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

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 (eg. 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.

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.

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.

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.

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.

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.

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.

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.

Well , there you have it , now you know a little bit more about what we are up against . I really don't read between the lines when it comes to Billy's cancer , I tend to take it all in stride , and I really do believe that in the end it will all be looked back on and put in the box with all the other memories . Then again , I really can't believe that My Son has cancer , and look at this as a bad dream that we'll one day awake from .

Thank you for listening ,
Billy's Dad

DISCLAIMER: This has been written for educational purposes only, it can not be used for diagnosing or treating any health problem . If you have or suspect you may have a health problem you should consult your doctor as soon as possible.