Ewings Sarcoma: Causes, Symptoms & Treatment

Growing pains are usually not a cause for concern in children. However, if the pain occurs repeatedly not only after activities but also at rest, a doctor should be consulted. Ewing’s sarcoma may cause this discomfort.

What is Ewing sarcoma?

First described by James Ewing, Ewing’s sarcoma is a form of bone cancer that most often develops in the thigh bones or pelvis. Less commonly, it affects the ribs. However, sarcoma can affect all other bones of the human skeleton. The extremely malignant tumor occurs mainly in children and adolescents between the ages of 10 and 25, with boys being affected much more frequently than girls. A characteristic feature of Ewing’s sarcoma is its rapid growth and thus the danger of spreading unhindered to other bones and the lungs. If left untreated, Ewing sarcoma leads to death within a few months.

Causes

No cause has yet been found for the development of Ewing sarcoma. Assumptions that inherited factors or previous excessive exposure to radiation from radiation therapy are causative for the occurrence of the tumor have not been confirmed. However, certain recurrent gene mutations on chromosome 22 could be linked to the tumor. However, inheritance of this gene mutation has not been demonstrated, so it remains unclear what causes the growth of malignant Ewing sarcoma.

Symptoms, complaints, and signs

The symptoms of Ewing’s sarcoma are nonspecific, as they can also occur in other diseases or in growth disorders. Initially, those affected often complain of recurrent pain, which usually increases with exertion. However, they persist at night. The pain may first appear after an insignificant trauma. Because the condition affects adolescents, it is often initially thought to be growing pains or pain from injury. Swelling and redness occur at the affected area. Sometimes, there may even be signs of failure if the spine or peripheral nerves are involved in the disease process. The larger the tumor becomes, the more often functional limitations occur. At this stage, metastases are often not yet present. However, if such general symptoms as fever, weight loss or fatigue are already present, this may indicate that metastases are already present. With early treatment before metastases form, there is a cure rate of approximately 65 percent. However, even after years, there is still a risk of recurrence if the cancer has not been completely eliminated by therapy. Nevertheless, even patients who are completely tumor-free often continue to suffer from various symptoms for a long time as a result of the therapy. The prognosis is worse in patients with metastases. The symptoms that occur in them depend on the organs affected by the metastases.

Diagnosis and course

Ewing’s sarcoma causes the patient to experience pain and swelling in the affected area of the body. The pain persists even at rest and usually occurs irregularly. Therefore, they are often misinterpreted and not associated with serious disease. When the tumor reaches a certain size, the patient experiences restricted movement and sometimes redness of the affected area, which ultimately leads him or her to the doctor. In a quarter of those affected, metastasis has already occurred at this stage and the cancer has been able to spread to other regions of the body. The diagnosis of Ewing’s sarcoma is initially based on blood tests, which can be confirmed by imaging techniques such as X-ray and computed tomography (CT) or magnetic resonance imaging (MRI). A biopsy is used to determine the exact nature of the sarcoma. This is the removal of a tissue sample of the tumor. Possible metastases in the lungs are searched for using imaging techniques. To determine whether the bone marrow has already been affected by Ewing’s sarcoma, a bone marrow puncture is necessary.

Complications

The tumor of Ewing sarcoma grows rapidly and often even affects the lungs. If left untreated, death can be expected within a few months. However, those who decide to seek medical and drug treatment early have a very good chance of a prompt and complete recovery.In most cases, Ewing’s sarcoma occurs in children who are in the growth phase. In this case, patients suffer from pain at rest that occurs even without movement. There is often fever and pain in the bones and joints, even if they are not moved. Swelling and redness may also develop there. In the pronounced stage, Ewing’s sarcoma leads to restrictions in movement and thus in everyday life. The affected person feels tired and cannot perform physical activities. If Ewing’s sarcoma is not treated, death may result. Therefore, treatment is always based first on the removal of the tumor, after which chemotherapy is advised. Unfortunately, there is no guarantee that the tumor will not recur. It is not uncommon that the patient has to repeat the treatment. In the case of repeated disease, the chances of cure are much lower than in the case of first-time disease. If the Ewing’s sarcoma cannot be treated, the pain is relieved so that the patient can go about his or her daily life in the usual way. Life expectancy is drastically reduced due to the disease.

When should you go to the doctor?

Ewing’s sarcoma is an explicit type of bone cancer that mostly affects young men between the ages of 10 and 25. Affected individuals usually complain of limb pain, so there may be stabbing pain even at rest. A doctor should be consulted if certain parts of the body hurt for no apparent reason. Only a medical and thorough examination can identify Ewing’s sarcoma. Early diagnosis is also very important for the later course of the disease, because only then can a full recovery take place. If a visit to the doctor is put off, the bone cancer can affect vital organs and thus lead to death.

Treatment and therapy

Treatment of Ewing’s sarcoma involves first trying to surgically remove the tumor. The patient then receives radiation therapy and chemotherapy. Due to the rapid growth of tumor cells, metastases often occur even after removal of the original tumor, which can be avoided by this approach. Radiation therapy is used either during or between two phases of chemotherapy. The total duration of treatment for Ewing’s sarcoma is 10 to 12 months. Despite modern and constantly improving therapy methods, relapses occur in 30 to 40 percent of patients. In the case of these so-called relapses, the chances of a cure are much lower than in the case of the initial disease, because there is currently no standardized treatment. Attempts are made to remove the tumor again surgically or to reduce its size by combined chemotherapy and radiation therapy. Often, particularly high-dose chemotherapy is also used. If all therapeutic measures remain unsuccessful, palliative care is used to try to relieve the patient’s pain. The worldwide analysis of treatment protocols aims to optimize the currently possible therapeutic measures and to improve the chances of survival of patients suffering from Ewing’s sarcoma, even in the long term.

Outlook and prognosis

The prognosis of Ewing sarcoma depends on the location of the tumor, the initiation of treatment, and its responsiveness to the chemotherapeutic agents used. A good response to chemotherapeutic agents occurs when the residual tumor mass contains less than ten percent live tumor cells. If more than ten percent of living tumor cells remain, this is evidence of a poor response to chemotherapy. Furthermore, the prognosis of the disease is much better if no metastases are present at the start of treatment. It has also been observed that the 5-year survival rate is higher when limb bones are affected (60-70 percent) than when pelvic bones are affected (40 percent). The possibility of complete surgical tumor removal also plays a role in prognosis. If the tumor has not yet spread and at the same time is very amenable to surgery, there is obviously a very good chance of complete cure. Non-operable tumors are irradiated. In this case, it is not possible to predict for the individual patient on the basis of statistical data whether he or she can be cured. This is true even for patients who have already developed metastases. The statistical data only reflect the probability.In addition, therapy optimization studies are constantly being conducted in order to achieve an improvement in prognosis even in advanced stages of the disease. However, it must also be noted that even completely tumor-free patients are often not symptom-free due to undesirable side effects of therapy.

Prevention

There are no direct preventive measures against Ewing sarcoma. However, recurrent pain should always be taken seriously in children and adolescents because this tumor grows extremely rapidly and can spread throughout the body via metastases. The earlier Ewing’s sarcoma is detected, the faster targeted treatment can be given.

Follow-up

After successful treatment of Ewing sarcoma, regular follow-up examinations ensure that new tumor formation or metastases to other organs can be detected and treated early. Late effects of chemotherapy or radiation therapy can also be detected in time. Each follow-up appointment includes a detailed physical examination, during which the region around the healed tumor in particular is carefully examined. Depending on the need, additional X-ray examinations, a computer tomography (CT), a magnetic resonance imaging (MRI) or ultrasound examinations are performed. Occasionally, skeletal scintigraphy may also be necessary. In general, Ewing sarcoma patients are recommended to have an X-ray of the lung every two months for the first two years and an X-ray or MRI of the primary tumor region every four months. In the third year, the intervals are extended to three and six months, respectively, and in the fourth year, a biannual radiological check of the lung is useful to exclude pulmonary metastases. From the fifth year onwards, this is only necessary once a year. Since chemotherapy can have late effects on the heart, the heart function should be checked once a year for ten years by means of an electrocardiogram (ECG) and heart ultrasound (echocardiography). If there are no symptoms, a routine examination every two years is sufficient. Annual blood and urine tests to check kidney function are also recommended for the first three years.

What you can do yourself

Ewing’s sarcoma is a form of bone cancer that particularly affects children and adolescents. The earlier the disease is discovered and therapy is started, the greater the chances of cure. Parents should therefore consult a doctor promptly if their children complain of certain symptoms and not play them down. In most cases, a bone tumor makes itself felt through pain in the affected area. Often, the skin and tissue over the diseased bone area is also swollen or inflamed. If parents observe such symptoms in their children, a doctor should always be consulted as a precaution, even if the causes are harmless in most cases. Not every bruise hides a fatal disease, but precautionary measures are still advisable. Even if children and adolescents constantly complain about aching arms or legs, this should not simply be dismissed as “growth pains“, but should be taken seriously and clarified by a doctor. If bone cancer is actually diagnosed, the young patients usually suffer greatly from the side effects of chemotherapy. Parents and children with the disease should seek contact with others affected in order to better cope with the psychological stress associated with the disease. Self-help groups are now active not only locally, but also on the Internet. If necessary, psychological support is also indicated.