Bone cancer in children and adolescents: Ewing's sarcoma
Primary malignant bone tumors, so-called sarcomas, represent a rare cancer. Certain aggressive tumors of this type of cancer, the so-called Ewing sarcomas, mainly occur in children and adolescents. The symptoms are often unspecific, which makes diagnosis difficult. If Ewing sarcoma and possibly also metastases are identified using imaging techniques and a biopsy, rapid therapy according to the latest standards is essential for survival. Today's treatment methods enable improved prognoses and chances of recovery.
A brief overview
The most important facts about Ewing sarcoma are briefly summarized in the following overview. Detailed information on this rare, serious illness can be found in the following article.
- definition: Ewing sarcomas are the second most common form of rare primary malignant bone tumors in childhood and adolescence. This term is used to summarize various aggressive, highly malignant and metastatic tumors that arise from primitive cells.
- Symptoms: The typical complaints are rather unspecific and are manifested by pain and swelling in the affected bone area. The origin of the disease is most often in the long bones, but all bones can be affected. In addition, the tumor tends to form metastases. If this is the case, the typical signs of cancer often appear.
- causes: The development of these sarcomas is still unknown. It is believed that primitive cells with a specific chromosome translocation trigger tumor growth. In most cases, the tumor forms in the (growing) bone tissue, rarely in the soft tissues.
- diagnosis: Diagnostic imaging methods are used in diagnosis, which in many cases reveal Ewing's sacromia due to specific structures. In any case, the diagnosis must be confirmed by a biopsy. Further examinations serve the search for possible metastases and the differential diagnosis.
- treatment: Today's standard therapy consists of induction chemotherapy, local therapy using surgery and / or radiation, and a final adjuvant chemotherapy. The treatment of a recurrent tumor is fundamentally more difficult.
- forecast: According to statistics, if there is only a local tumor without metastases, long-term cure is currently expected in around 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: The latest findings have significantly improved the prognoses and chances of survival. The serious illness is still part of many research approaches in order to be able to offer those affected ever better treatment options.
Sarcomas are rare malignant cancers that can originate in connective and supporting tissues (bones, cartilage and fat) or in muscle tissue. The so-called Ewing sarcoma (also Ewing sarcoma) describes a primary malignant bone tumor that mainly occurs in children and adolescents. In addition to osteosarcoma, Ewing sarcomas are the second most common bone tumor in young age. Such a sarcoma is diagnosed in about ten to fifteen percent of all malignant bone tumors. The disease manifests itself particularly between the ages of ten and twenty years, with the peak at twelve to seventeen years. The disease is less common in younger children, infants or in (young) adulthood. A little more boys than girls tend to get sick.
According to the current criteria of the WHO (World Health Organization), various tumors are grouped under the Ewing sarcomas, which were originally classified separately. The term therefore includes all sarcomas described so far from the group of Ewing tumors, the askin tumors of the chest wall, the atypical Ewing sarcomas and all forms of malignant peripheral neuroectodermal tumors (PNET or MPNET). Ewing sarcomas are aggressive (highly malignant) and metastatic tumors that arise from primitive, small, round cells. These can be colored blue in diagnostics ("small blue cells").
The pathogen was named after the American pathologist James Ewing (1866-1943), who was involved in cancer research, among other things. Existing preparatory work and descriptions of similar tumors by Georg Albert Lücke (1829-1894) and Otto Hildebrand (1858-1927) made him achieve a medical breakthrough in 1920 by describing what was then a new type of bone tumor.
It is important to differentiate a bone sarcoma from a bone infection or from inflammation of the bone (osteitis) or inflammation of the bone marrow (osteomyelitis).
The typical complaints are unspecific and manifest themselves primarily through pain and possibly also a swelling in the bone area that can be felt. In general, all bones can be affected, but the most common form of cancer is in the bone shaft (diaphysis) of the long long bones of the thigh and upper arm, as well as in the pelvis, ribs and clavicle.
The location and type of pain depends on which areas are affected. For example, if the tumor is in the pelvis, deep low back pain or back pain may occur. If the place of origin is in the ribs, flank pain can occur. The pain can also occur intermittently and increase under stress.
Due to the spread of the tumor, infested bones become unstable and bone fractures can result. In addition, typical signs of cancer in general, such as lymph node swelling, night sweats, fever, decreased performance and weight loss, may also appear. If these symptoms occur, the focus of the disease is often already relocated to other parts of the body. In about a quarter of the sufferers, metastasis (formation of daughter tumors) is already detected during the initial diagnosis. Metastases are often found in the lungs or other bones and in the bone marrow.
The exact origin of Ewing sarcoma is still unclear. The disease is believed to originate from primitive cells that have a certain genetic abnormality. This is usually a chromosome mutation or a translocation between chromosomes 11 and 22. The new gene that is created is called EWS / FLI1 and its function probably leads to cancer formation.
A genetic predisposition is unlikely, but it is striking that almost no cases of illness are known to the African and Chinese population. Since the tumor mainly forms in adolescents, it is reasonable to assume that growing bone is preferred.
In the vast majority of cases, this type of cancer is of bony origin, but less often (and more likely in older age), an Ewing tumor can also occur extraosseously - as a so-called soft tissue sarcoma.
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 presumption of a bone disease, X-rays are usually first taken. A bony Ewing sarcoma can be seen on the X-ray images due to bone damage similar to moth-eaten bone, possibly also with tissue surrounded by infiltration. The periosteum can also be affected, which can be seen in various periosteal reactions. Examples are the onion skin-like lifting of several lamellae, the periosteal spur (Codman triangle) and so-called spicules as structures similar to the sunbeam.
With the help of other imaging methods (computed tomography and magnetic resonance tomography), it is often possible to make more precise statements about the tumor and the soft tissue.
If, based on these examinations, there is suspicion of an Ewing sarcoma, a tissue sample of the tumor is always taken for further examinations to confirm the diagnosis (biopsy). With regard to subsequent 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 sarcomas. The later surgeon should always be included in the diagnosis at an early stage in order to minimize the risk of complications.
In tumor tissue examination, immunohistochemical (antibody staining) and morphological findings, possibly supplemented by the genetic detection of a chromosome translocation, allow a reliable statement about the presence of the disease. The results also ensure a differential diagnosis, such as other small, blue, round cell tumors or osteomyelitis. Immunohistochemically, Ewing sarcoma gives a positive stain (blue) for the antibody O13 (CD99).
Since this type of tumor often spreads through metastases, a spread diagnosis (staging) follows immediately after the initial diagnosis, in which the most common locations of metastases are first checked. Imaging methods are used again, for example to examine the lungs. Bone scintigraphy, bone marrow biopsy and complementary bone marrow aspiration are also carried out to detect tumor cells. A lumbar puncture may be an option. The advantage of an additional nuclear medical whole-body positron emission tomography (PET) is still being discussed.
Ewing sarcomas are extremely aggressive, highly malignant tumors, from which the vast majority of those affected would die without appropriate therapy. Medical advances in treatment have made it possible for the disease to be more treatable and even curable under certain conditions. 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 proven to be the most effective so far. The standard procedure initially provides for neoadjuvant chemotherapy (induction chemotherapy), which prevents metastasis and is intended to enable further treatment by reducing the tumor mass. Local therapy then involves surgical removal and / or radiotherapeutic treatment of the tumor and possible metastases. At the end of the therapy, adjuvant chemotherapy is to be used to kill cancer cells that may still have remained in the body.
So far, often in combination, the substances actinomycin D, cyclophosphamide, doxorubicin, etoposide, ifosfamide and vincristine have been used for drug therapy. There are often serious side effects, especially in older people. The use of high-dose chemotherapy with stem cell transplantation, bisphosphonates and other methods are still being tested.
Bone defects arising during the operation can be treated afterwards using various reconstruction methods, such as (endo) prostheses. The treatment, which can last up to a year, is often followed by rehabilitation.
Ewing sarcomas show a tendency to recur, which is an important aspect in treatment. On the basis of various criteria (Ewing 2008 study), those affected are now divided into three recurrence risk groups according to the standard procedure, from which the respective intensity of chemotherapy (number of cycles) is derived
The relatively high recurrence rate requires regular follow-up examinations, in which not only the emergence at the same place of origin but also possible metastasis formation are checked. Treatment for a recurrence is usually the intensification of chemotherapy. Other approaches are still in the test phase.
Because of their aggressiveness and rapid metastasis, sarcomas require special conventional medical treatment. As with any other cancer, biological cancer therapy can help to strengthen the body and mind. In consultation with the treating doctor and taking into account the individual course of the disease, various naturopathic treatments can support the healing process.
If it is exclusively a local tumor without metastases, there is a rather positive prognosis for the first disease. If this is the case, around 65 percent of those affected can currently be cured over the long term. In about fifteen percent of those affected, however, metastases already occur during the initial diagnosis, which worsens the chances of recovery. According to statistics, a five-year survival rate is then assumed for an average of a quarter of those affected. If only the lungs are affected, there is usually a better chance of spreading to other parts of the body.
If the cancer recurs, the prognosis deteriorates similarly unfavorably. The most important factor in this connection is the period between the initial diagnosis and the recurrence. Depending on the patient's case, the chances of a cure may differ 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 provide, among other things, the current standard of care. The new therapeutic approaches have shown that the long-term cure for patients with locally limited Ewing 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 few restrictions.
Many clinical trials are ongoing for further improvements in therapy and new treatment options. For example, the new drugs irinotecan and temozolomide are being tested, which are effective in combination and particularly with recurrent Ewing sarcomas. Researchers recently announced this in the journal Acta Oncologica.
Further work focuses on the research and allocation of tumors that are very similar to Ewing sarcoma (Ewing-like sarcomas). (tf, cs)
For further reading:
Bone marrow inflammation
Author and source information
This text corresponds to the specifications of the medical literature, medical guidelines and current studies and has been checked by medical doctors.
Dr. rer. nat. Corinna Schultheis
- Grevener, Knut / Haveman, Lianne M. / Ranft, Andreas / et al .: Management and Outcome of Ewing Sarcoma of the Head and Neck; Pediatric blood & cancer, Volume 63, Issue 4, April 2016, Pediatric blood & cancer
- Society for Pediatric Oncology and Hematology (GPOH): S1 guideline for Ewing sarcomas of children and adolescents, status: June 2014, detailed view of guidelines
- Amboss GmbH: Malignant bone tumors (accessed: June 26, 2019), amboss.com
- Mayo Clinic: Ewing sarcoma (accessed: June 26, 2019), mayoclinic.org
- National Cancer Institute (NCI): Ewing Sarcoma Treatment (accessed: June 26, 2019), cancer.gov
- American Academy of Orthopedic Surgeons: Ewing's Sarcoma (accessed: June 26, 2019), orthoinfo.aaos.org
- UpToDate, Inc .: Clinical presentation, staging, and prognostic factors of the Ewing sarcoma family of tumors (accessed: June 26, 2019), uptodate.com
- National Health Service UK: Ewing sarcoma (accessed: June 26, 2019), nhs.uk
- U.S. National Library of Medicine (NLM): Ewing sarcoma (access: June 26, 2019), ghr.nlm.nih.gov
- Ranft, Andreas / Seidel, Corinna / Hoffmann, Christiane / ua: Quality of Survivorship in a Rare Disease: Clinicofunctional Outcome and Physical Activity in an Observational Cohort Study of 618 Long-Term Survivors of Ewing Sarcoma, Journal of Clinical Oncology, 2016, ascopubs .org
ICD codes for this disease: C41ICD codes are internationally valid encodings for medical diagnoses. You can find yourself e.g. in doctor's letters or on disability certificates.