Ewing's sarcoma is a dangerous oncological disease in which cancer cells develop in bone tissue. Most often (in 70% of cases) this disease affects the bones of the lower extremities. Ewing's sarcoma code according to ICD - 10 C40, C41.
The disease got its name from the name of the doctor James Ewing, who discovered this pathology in 1921. In his writings, James described the disease as a tumor that occurs predominantly in tubular long bones.
Ewing's sarcoma: what is it?
Of all the existing oncological diseases, this pathology is considered one of the most dangerous. This is explained by the high aggressiveness of the tumor: it progresses rapidly and gives multiple metastases.
The main group of patients with this diagnosis are children aged 10-20 years. In adults, Ewing's sarcoma is also diagnosed, but much less frequently. This type of tumor is more common in boys than in girls. To date, doctors cannot explain this gender dependence.
Clinical and morphological symptoms of bone cancer are very similarwith the manifestation of a round cell tumor of soft tissues, the only difference is the localization of pathological cells. For this reason, a tumor affecting the skeleton is called classic Ewing's sarcoma, and one that occurs in the soft tissues of the chest, spine and retroperitoneal space is called extraskeletal sarcoma.
Causes of disease
The exact reasons for the development of this type of sarcoma could not be found. However, leading oncologists put forward a number of assumptions based on the analysis of disease statistics.
- Genetic predisposition. Ewing's sarcoma in children is often diagnosed in several sisters and brothers, which led to the theory of a predisposition to bone sarcoma.
- Bone injuries. In about 40% of cases, the tumor focus is located in the place where the injury was previously (fracture, severe bruising).
- Some skeletal anomalies. The presence of skeletal abnormalities, including aneurysmal bone cyst and enchondroma, has been suggested to affect the risk of developing sarcoma.
- Pathologies and anomalies in the genitourinary system. Among them: reduplication of the renal system or hypospadias.
It is important to note that the presence of one or another factor does not at all mean that a person will develop a cancerous tumor over time. The presence of these signs only increases the risk of developing the disease.
Risk factors
There are also several risk factors that require increased attention:
- male - Ewing's sarcoma is much less common in girls and women;
- ages 10-20 - mostpatients (64%) belong to this age group;
- race - white skinned people suffer from bone sarcoma much more often than blacks.
Pathogenesis (what happens during illness)
The tumor in this cancer is represented by small rounded cells that have divisions in the form of fibrous layers. The distinctive features of the cell structure include the correct shape, the presence of oval or rounded nuclei with basophilic nucleoli and chromatin. The tumor does not produce new bone tissue.
What is Ewing's sarcoma and how does it develop? The growth of the neoplasm is mainly due to uncontrolled cell division. According to scientists, such a pathology is provoked by a special type of genetic damage. Among such damages is a mutation of the gene responsible for encoding the production of a receptor capable of transforming a growth factor. As a result, the increased division of pathological cells is not blocked.
Types of disease
In medicine, Ewing's sarcoma is divided into 2 types:
- localized - a neoplasm that affects a certain area of bone tissue and possibly spreads to nearby lymph nodes and tendons;
- metastatic - a sarcoma that has spread throughout the body and affected the bones, lymphatic system, blood vessels, and distant internal organs.
Stages of disease progression
In addition, for the convenience of differentiation,division into stages. When making a diagnosis, this indicator must be indicated, as this gives doctors the most complete picture of the disease.
- 1 stage. The very beginning of the development of the disease. Pathological cells at this time are located only on the surface of the bones and do not penetrate deep into. The size of the tumor reaches only a few millimeters. It is quite simple to get rid of the neoplasm at this time, however, the disease at stage 1 is extremely rare. The reason is the absence of symptoms.
- 2 stage. Ewing's sarcoma increases in diameter and at the same time begins to penetrate into the deeper layers of bone tissue. The prognosis of treatment in this case is also optimistic. With proper treatment, about 60-65% of patients are cured of pathology.
- 3 stage. Not only bone tissue is involved in the oncological process, but also nearby soft tissues, lymph nodes located nearby. The chances of successful treatment are somewhat lower - about 40%.
- 4 stage. Sarcoma spreads throughout the body and gives rise to secondary foci of cancer. A 5-year survival rate for Ewing's sarcoma and achieving remission is possible in 7-10% of cases. With good tumor sensitivity and taking into account bone marrow transplantation at stage 4, it is possible to increase the patient's chances up to 30%.
An important difference between Ewing's sarcoma and many other types of cancer can be called the rapid development.
Clinical picture (symptoms)
In the early stages of the development of Ewing's sarcoma, symptoms are practically not manifested, which prevents early diagnosis of the disease. Moreover, when describing the symptoms, one should keep in mind: ineach case, the symptoms may vary slightly depending on the location of the tumor.
Pain. Painful sensations can be included among the first symptoms that appear in bone tumors. Initially, the pain is characterized as short-term non-permanent. In the course of the progression of the disease, they increase, however, patients often mistake such symptoms for manifestations of other pathologies. Unlike pain of an inflammatory nature, pain in Ewing's sarcoma does not subside at rest and with rigid fixation of the limb. Especially often, patients complain of increased pain at night.
Immobilization of the joint. Approximately 3-4 months after the first manifestations of symptoms, the adjacent joint begins to function poorly. This is manifested in the difficulty of flexion and extension of the limb, the appearance of pain up to complete immobilization.
Redness and pain in the soft tissues. A progressive bone tumor gradually involves the process and soft tissues located in close proximity. The skin in this area becomes reddened, there is some swelling. During palpation, patients note soreness of the tissues.
Increased lymph nodes. At stages 1-2 of sarcoma, only those lymph nodes that are located near the tumor can increase. At stages 3 and 4, there is an increase and soreness of the cervical, inguinal and other lymph nodes.
Deterioration of the general condition. When moving to the stage of metastasis, patients experience a sharp weight loss, lack of appetite, complete apathy, rapidfatigue. Possible increase in body temperature, fever.
Characteristic symptoms
There are also some symptoms of Ewing's sarcoma, which are characteristic only when certain bones are affected:
- bones of the pelvis and lower extremities - development of lameness;
- spine - damage to the spinal cord, radiculopathy, the appearance of delayed reflexes and muscle inhibition;
- chest - blood in sputum, shortness of breath, respiratory failure.
Lab tests
During the diagnostic procedures, doctors pursue several goals at once: to identify the focus of the disease and its nature, as well as to check for the presence of metastases in other organs and tissues. To do this, several types of laboratory tests and hardware diagnostics are used at once.
Blood test. When studying the quantitative composition of blood, doctors take into account the indicators of erythrocytes, platelets and leukocytes. Differences from the norm indicate pathological processes in the body and the spread of the tumor.
Biopsy. This medical term refers to the removal of a sample of tumor tissue or bone marrow. This procedure is carried out using a long needle. Only after a histological examination of these tissues can doctors confirm the malignant nature of the neoplasm.
Hardware diagnostics
Thanks to hardware diagnostic methods, doctors receive information about the size of the neoplasm, its exact localization andthe number of outbreaks.
X-ray. X-rays are considered one of the most important ways to diagnose bone cancer. If it is necessary to check the condition of the internal bone substance, then a contrast solution is used with x-rays. He althy bone is gray on x-ray, while cancerous bone is dark.
Computed tomography. This procedure determines the size of the tumor and the presence (or absence) of metastases.
Magnetic resonance imaging. It acts as an effective diagnostic tool.
Chemotherapy treatment
There are several methods of treatment at once. The choice of one or another approach is based on the stage of the disease, the location of the tumor, comorbidities and the age of the patient.
At the same time, the treatment of Ewing's sarcoma follows a slightly different scenario than the treatment of other types of cancer. While many other forms of cancer require surgery in the first place, sarcoma is more commonly treated with chemotherapy. Conducting such a course before surgery helps to reduce the size of the tumor.
The principle of chemotherapy is the use of very strong drugs. They can be presented in different forms (tablets, injections). Once in the human body, these medicinal substances have a detrimental effect on cancer formation, destroy the structure of its cells and block the development of Ewing's sarcoma. A prognosis with a high chance of cure is possible if no more than 5% of the tumor remains in the patient's body after chemotherapy.
This treatment is carried out inseveral approaches every 2 or 4 weeks. The most common drugs used in chemotherapy are: vepezid, phosphamide, cyclophosphamide, vincristine, etoposide and some others.
Surgery
Most often, surgical operations are included in complex treatment. After reducing the size of the tumor under the influence of chemotherapy, surgeons remove the affected part of the tissues (soft and bone). When large volumes of bone are removed, this area is replaced with grafts. Very rarely, a limb amputation (or partial amputation) is required to eliminate the tumor.
If necessary, an additional course of chemotherapy or radiotherapy is prescribed after the operation. This will make it possible to destroy the remnants of pathological cells (if any) and thereby prevent the recurrence (re-spread) of cancer.
Radiation therapy
Beam or radiotherapy is another effective way to deal with Ewing's sarcoma. During this procedure, the patient's body is exposed to radiation that has a destructive effect on cancer cells. This method of eliminating pathology is often used in the postoperative period to destroy those cancer cells that remain in the patient's body.
Each of the listed methods of therapy patients experience quite hard. After surgery, this is a long recovery period, after radiation and radiotherapy there is a period of side effects. This is explained by the fact that radio emission and powerful drugs have a negative effect not only on cellstumors, but also on absolutely he althy tissues (only in a smaller volume). At this time, patients may experience frequent bouts of nausea and vomiting, a sharp drop in immunity, an increased risk of contracting various diseases, and disruption of many body systems.
At the same time, properly prescribed complex treatment allows you to get rid of even such a terrible and serious disease as Ewing's sarcoma. How long such patients live depends on individual characteristics, but a positive effect can be achieved even at stage 4 of the disease.
