Ewing's sarcoma - causes, symptoms and chances of recovery

Bone cancer in children and adolescents: Ewing's sarcoma

Primary malignant bone tumors, so-called sarcomas, represent a rare form of cancer. Certain aggressive tumors of this type of cancer, the so-called Ewing's sarcoma, mainly occur in children and adolescents. The symptoms are often nonspecific, making diagnosis difficult. If Ewing's sarcoma and possibly also metastases are detected by means of imaging techniques and a biopsy, rapid therapy according to the latest standards is essential for survival. Today's treatment methods enable improved prognosis and chances of recovery.

contents

• Bone cancer in children and adolescents: Ewing's sarcoma
• A short overview
• definition
• symptoms
• causes
• diagnosis
• treatment
• Naturopathic treatment
• forecast
• Current state of research

A short overview

Key facts about Ewing's sarcoma are summarized in the following overview. Detailed information on this rare, severe disease can be found in the following article.

• definition: Ewing's sarcoma represents the second most common form of rare primary malignant bone tumors in childhood and adolescence. This term covers various aggressive, highly malignant and metastatic tumors that arise from primitive cells.
• symptoms: The typical symptoms are rather unspecific and are expressed by pain and swelling in the affected bone area. The most common is the origin of the disease in the long bones, but all bones can be affected. In addition, the tumor tends to metastasis. If this is the case, the typical signs of cancer often occur.
• causes: The formation of these sarcomas is still unclear. It is believed that primitive cells with a certain chromosomal translocation trigger tumor growth. In most cases, the tumor forms in the (growing) bone tissue, rarely in the soft tissues.
• diagnosis: Diagnosis involves the use of imaging techniques that in many cases reveal an Ewing sacral due to specific structures. In any case, the diagnosis must be backed up by a biopsy. Further investigations serve the search for possible metastases and differential diagnosis.
• treatment: Today's standard therapy is composed of an induction chemotherapy, a local therapy by means of surgery and / or radiation and a final adjuvant chemotherapy. The treatment of a recurrent tumor is in principle more difficult.
• forecastIf there is only a local tumor without metastases is currently expected, according to statistics, a long-term cure in about 65 percent of those affected. If metastases have already formed or if the cancer recurs after successful treatment, this significantly worsens the prognosis.
• Current state of research: Recent findings have significantly improved the prognosis and chances of survival. The serious illness continues to be part of many research approaches in order to be able to offer patients ever-better treatment options.

If children or adolescents have malignant bone cancer, up to fifteen percent of the cases are Ewing's sarcoma. (Image: Photographee.eu/fotolia.com)

definition

Sarcomas are rare malignant cancers that may have their origin in the connective and supporting tissue (bone, cartilage and adipose tissue) or in muscle tissue. The so-called Ewing sarcoma (also Ewing's sarcoma) refers to a primary malignant bone tumor, which occurs mainly in children and adolescents. In addition to osteosarcoma, Ewing's sarcoma is the second most common bone tumor in young age. In about ten to fifteen percent of all malignant bone tumors such a sarcoma is diagnosed. The disease manifests itself in particular between the ages of ten and twenty years, with the peak at twelve to seventeen years. Less commonly, the disease occurs in younger children, infants or in (young) adulthood. The tendency is for slightly more boys than girls.

According to the current criteria of the WHO (World Health Organization), among the Ewing sarcoma different tumors are summarized, which were originally classified separately. The term thus includes all sarcomas described so far from the group of Ewing tumors, the Askin tumors of the thoracic wall, the atypical Ewing's sarcoma and all forms of malignant peripheral neuroectodermal tumors (PNET or MPNET). Ewing's sarcomas are aggressive (highly malignant) and metastatic tumors that arise from primitive, small, round cells. These can be colored blue in the diagnostics ("small blue cells").

The name of the disease was the American pathologist James Ewing (1866-1943), who was involved in, among other things, cancer research. Existing preparatory work and descriptions of similar tumors by Georg Albert Lücke (1829-1894) and Otto Hildebrand (1858-1927), made him in 1920 with the description of the then new type of bone tumor to achieve a medical breakthrough.

Important is the demarcation of a bone sarcoma from a bone infection or from a bone inflammation (osteitis) or osteomyelitis (osteomyelitis).

The two most common primary malignant bone tumors in infancy, osteosarcoma and Ewing's sarcoma, are characterized by aggressive tumor growth with metastasis. (Image: designua / fotolia.com)

symptoms

The typical complaints are nonspecific and are mainly expressed by pain and possibly also by a swelling in the bone area, which can be palpated. In general, all bones may be affected, but most commonly the cancer is found in the bone shaft (diaphysis) of the long bones of the thigh and upper arm, as well as in the pelvis, in the ribs and in the collarbone.

The location and type of pain depends on which areas are affected. For example, if the tumor is in the pelvis, it can cause deep back pain or backache. If the place of origin lies in the ribs, it can come to flank pain. The pain can also occur intermittently and increase under stress.

As the tumor spreads, affected bones become unstable and fractures can result. In addition, typical signs of cancer can also occur in general, such as lymphadenopathy, night sweats, fever, decreased performance and weight loss. If these symptoms occur, there is often already a location of the disease in other parts of the body. In about one quarter of the patients is detected at the first diagnosis of metastasis (formation of secondary tumors). Metastases are often found in the lungs or in other bones and in the bone marrow.

causes

The exact genesis of Ewing's sarcoma is still unclear. It is believed that the disease originates from primitive cells that have a certain genetic abnormality. This is usually a chromosome mutation or a translocation between chromosomes 11 and 22. The resulting gene is called EWS / FLI1 and probably leads to cancer formation in its function.

There is no presumption of genetic predisposition, but it is striking that almost no disease is known in the African and Chinese populations. Since the tumor forms mainly in adolescents, it seems likely that preferentially growing bone substance is affected.

In most cases, this cancer is of bony origin, less often (and more in the higher age of onset), an Ewing tumor but also extraosseous - as a so-called soft tissue sarcoma - occur.

For a reliable diagnosis, a biopsy is used in each case after imaging procedures. (Image: science photo / fotolia.com)

diagnosis

Due to the non-specific symptoms and possible confusion with other diseases such as bone inflammation, there is often a delay in the diagnosis.

If there is a suspicion of a disease of the bones, radiographs are usually made first. On the X-ray images, a bony Ewing's sarcoma can be detected by moth-like bone damage, possibly also with infiltration of surrounding tissue. The periosteum may also be affected, as shown by various periosteal reactions. Examples are the onion peel-like detachment of several lamellae, the periost spur (Codman triangle) and so-called spiculae as sunbeam-like structures.

With the help of other imaging techniques (computed tomography and magnetic resonance tomography), more precise statements about the tumor and the soft tissue can often be made.

If there is a suspicion of Ewing's sarcoma as a result of these examinations, a tissue sample of the tumor is always taken for further examination (biopsy) to ensure the diagnosis. With regard to later therapeutic measures, it is particularly important that possible surgical access routes to the tumor are taken into account or used. A biopsy should therefore only be performed by doctors who specialize in sarcoma. The subsequent surgeon should always be included early in the diagnosis to minimize the risk of complications.

In tumor tissue examination, immunohistochemical (antibody staining) and morphological findings, possibly complemented by genetic evidence of chromosome translocation, provide a reliable indication of the presence of the disease. The results also ensure a differential diagnosis, for example to other small, blue, round-celled tumors or osteomyelitis. Immunohistochemical results in Ewing's sarcoma positive staining (blue) for antibody O13 (CD99).

Since this type of tumor often spreads via metastases, immediately after the initial diagnosis, a staging procedure follows, in which the most frequent locations of metastases are first examined. Again, imaging techniques are used, for example to examine the lungs. Bone scintigraphy as well as bone marrow biopsy and supplementary bone marrow aspiration for the detection of tumor cells are also performed. Under certain circumstances a lumbar puncture is possible. The benefit of additional nuclear medicine full body positron emission tomography (PET) is still under discussion.

The current standard of care includes special chemotherapy before and after additional surgery and / or radiation. (Image: tashatuvango / fotolia.com)

treatment

The Ewing's sarcomas are extremely aggressive, highly malignant tumors, in which the vast majority of those affected would die without appropriate therapy. Medical advances in treatment have made it possible to treat the disease better and, under certain conditions, even to cure it. The treatment of bone and soft tissue sarcomas follows the same procedure.

The combination of drug treatment (chemotherapy) and local therapy (surgery and radiation) has been the most effective so far. The standard procedure initially envisages neoadjuvant chemotherapy (induction chemotherapy), which should prevent metastasis and enable further treatment by reducing the tumor mass. Thereafter, local therapy results in surgical removal and / or radiotherapeutic treatment of the tumor and possible metastases. By means of adjuvant chemotherapy, cancer cells are to be killed at the end of the therapy, which may still have remained in the body.

So far, the substances actinomycin D, cyclophosphamide, doxorubicin, etoposide, ifosfamide and vincristine are often used in combination for drug therapy. Often there are serious side effects, especially in elderly sufferers. The use of high-dose therapies with stem cell transplantation, bisphosphonates and other methods are still being tested.

Bone defects resulting from the operation can be subsequently treated by means of different reconstruction methods, such as, for example, with (endo) prostheses. Often the treatment, which can last up to one year, is followed by a rehabilitation.

Ewing sarcomas show a tendency to recurrence, which is an important aspect of the treatment. Based on various criteria (Ewing 2008 study), patients are now classified according to the standard procedure into three recurrence risk groups, from which the respective intensity of the chemotherapy (number of cycles) is derived

The relatively high recurrence rate requires regular follow-up examinations, in which not only the origin at the same place of origin but also possible metastases are controlled. Treatment for recurrence is usually the intensification of chemotherapy. Further approaches are still in the test phase.

Naturopathic treatment

Because of their aggressiveness and rapid metastases, sarcoma requires a special conventional medical treatment. However, as with any other cancer, biological cancer therapy can help strengthen the body and psyche. In consultation with the attending physicians and taking into account the individual course of the disease, various natural remedies may possibly support the healing process.

In the most favorable cases without metastases, with the help of the latest therapies, a long-term cure (freedom from tumors) of about 65 percent of those affected is possible. (Image: pingpao / fotolia.com)

forecast

If it is exclusively a local tumor without metastases, there is a rather positive prognosis for the first disease. If this is the case, about 65 percent of those affected can currently be cured long-term. However, in about fifteen percent of those affected, metastases occur at the time of the first diagnosis, which worsens the chances of recovery. According to statistics, a five-year survival rate is assumed for an average of one quarter of the patients. If only the lungs are affected, there are usually better chances than with a further spread to other body regions.

If the cancer occurs again, the prognosis worsens similarly unfavorably. The most important factor in this context is the period between the first diagnosis and the recurrence. Depending on the patient case, however, the chances of recovery may deviate from the statistical values.

Current state of research

The latest medical findings have been summarized in the guideline "Ewing sarcomas of childhood and adolescence" and include the current standard of care. The new therapeutic approaches have shown that the long-term cure for patients with localized Ewing's sarcoma has improved to 60 to 70 percent. According to a study published in the Journal of Clinical Oncology, survivors can return to a largely normal life with only a few restrictions.

For further improvements in therapy and new treatment options, many clinical trials are taking place. For example, the new drugs Irinotecan and Temozolomid, which are shown to be effective in combination and especially in recurrent Ewing sarcomas, are being tested. This has recently been announced by researchers in the journal Acta Oncologica.

Further work is focused on the research and assignment of tumors that are very similar to Ewing sarcoma (Ewing-like sarcoma). (tf, cs; updated on 23.01.2019)

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