Ewing's sarcoma. Classification of bone tissue sarcomas: types and types. The patient's prognosis depends on

One of the most aggressive malignant diseases It's Ewing's sarcoma. This type of tumor was discovered at the beginning of the 20th century. But until now, the disease is considered poorly understood. The famous oncologist James Jung singled out this type of sarcoma in separate group due to the fact that it has some features.

Most often, the tubular bones, pelvis, and spine are affected by the tumor. Until recently, 90% of patients rapidly developed metastases that spread to the bone marrow, lymph nodes, or lungs. In addition, this rapidly growing tumor is difficult to diagnose. Therefore, Ewing's sarcoma has always been considered the most dangerous disease with a poor prognosis.

The complexity of its treatment is also in the fact that it most often affects children. The peak incidence occurs at the age of 10-15 years. But this tumor occurs from 5 to 30 years, and mainly in boys. Due to the fact that the complaints of the child are accepted mainly as the consequences of an injury or inflammatory diseases, the neoplasm is rarely diagnosed on initial stages. Therefore, his prognosis was always unfavorable. Although in recent times with the advent of modern methods of treatment, more than 70% of patients can lead full life after recovery.

A feature of Ewing's sarcoma is also that the location of the tumor depends on the age of the patient. In children and adolescents, the long tubular bones of the extremities are most often affected. Young people suffer from neoplasms in the pelvic bones, ribs, collarbones, and vertebrae. Very rarely, but only soft tissues, such as the lungs, are affected.

Cause of the disease

In spite of long years studies of the disease, the cause of the development of the tumor has not yet been determined. But scientists have identified several predisposing factors for the onset of the disease:

• first of all, it is a hereditary predisposition: Ewing's sarcoma most often develops in those whose relatives had sarcoma;
• with genetic abnormalities;
• congenital anomalies in the development of bones or the genitourinary system;
• bone injuries can become a trigger;
• the appearance of the tumor depends on age, sex and race: most often the disease occurs between the ages of 5 and 25, mainly in boys, in people with white skin.

Ewing's sarcoma is serious illness that needs to be treated in the clinic

How the disease manifests itself

The main symptoms of Ewing's sarcoma are bone pain. At first they can be mild, aching, interrupted for a while. Then they become more intense. Their peculiarity is that they do not subside, but only intensify at night or when the affected limb is immobilized. In this they differ from pain in injuries and inflammatory diseases.

Unpleasant sensations are aggravated by feeling the sore spot. In addition, the movement in the nearest joints is disturbed, the patient cannot sleep normally. Such pain is difficult to remove with conventional drugs.

Over time, the tumor captures the surrounding soft tissues. Developing severe swelling with hyperemia of soft tissues, which become compacted, hot to the touch. Symptoms of intoxication also appear: fever, nausea, weakness, weight loss, swollen lymph nodes, lack of appetite, anemia.

In the process of the development of the disease, its external signs are more and more manifest: the tumor grows, the skin above it becomes purple or cyanotic, thinner, veins begin to stand out strongly. Due to pathological processes in the bones, their fractures are often observed at the slightest load.

Other symptoms indicate the localization of Ewing's sarcoma: if the tumor develops in the lungs, it may be hemoptysis, shortness of breath. Its localization in the pelvic bones may indicate urinary incontinence, impaired bowel function, paralysis of the lower extremities. It is in the pelvic region that the disease occurs most often - in almost half of the cases. The long tubular bones of the limbs are often also affected. In this case, the disease is easier to detect by external signs: a change in the shape of the limb, lameness, impaired mobility.

Doctors distinguish several stages of the development of the disease, in accordance with which treatment is prescribed.

Stages of development of the disease

Ewing's sarcoma develops very quickly, so it is difficult to isolate in this process various stages. But doctors often classify diseases as follows:

• Stage 1 is a small tumor on the surface of the bone, outwardly invisible, the disease is almost asymptomatic;
• Stage 2 - the neoplasm grows in depth bone tissue, affects the surrounding muscles and tendons, appear severe pain, visible external changes in soft tissues;
• Stage 3 - metastases appear in the nearest organs and tissues, the tumor itself is already very noticeable, and pain interferes with sleep and movement;
• Stage 4 - distant metastases spread throughout the body, which greatly worsens the general condition of the patient.

Diagnosis of the disease

For a correct diagnosis, it is necessary to consult a doctor for examination if the child has alarming symptoms. Diagnosis of the disease includes the following methods:

• first of all, they do x-rays in the place where there is a suspicion of tumor development - this is the primary method for determining the presence of the disease;
• to confirm the diagnosis, an MRI of bones and soft tissues is shown, which will show the boundaries of the neoplasm, the degree of its spread, damage to nerves and blood vessels;
• computed tomography of the lungs is also done to determine the presence of metastases;
• sometimes prescribed osteoscintigraphy, ultrasound, angiography, positron emission tomography;
• for more exact definition the type of tumor is a biopsy, bone marrow examination, immunological and genetic tests.

Such an extensive diagnosis will help differentiate the disease from others like it. After all, the symptoms are often similar to the course of chronic osteomyelitis, neuroblastoma, osteosarcoma, lymphoma. This will allow more effective treatment to be prescribed.

Various diagnostic methods help to make the correct diagnosis in time

How to treat Ewing's sarcoma

This disease is very difficult to diagnose in the early stages. Usually, when the diagnosis is made, the tumor has already metastasized. But at timely treatment it is possible to completely eliminate the pathological focus, prevent metastases, and also exclude the possibility of relapses. But most often the prognosis for patients is unfavorable. Complete cure is possible only at the initial stage of the disease. In addition, the effectiveness of therapy depends on the location of the tumor. Worst of all, if metastases have spread to the bone marrow.

The most radical and effective treatment for this tumor is surgical removal. But the operation is not always shown. Therefore, conservative treatment of the disease is more common: chemotherapy or radiation. The prognosis of cure and survival of patients depend primarily on its timeliness. The sooner the patient comes to medical institution the sooner you can alleviate his condition. Tumor therapy must necessarily be carried out in a specialized clinic, where complex treatment will be prescribed, including several methods.

In many cases, only surgical treatment can save the patient's life.

Surgical removal of the tumor

This is the most effective method disease therapy. Depending on how far the tumor and its metastases have spread, not only the affected areas can be cut out during the operation. The pelvic or femur bones, ribs, and other parts of the skeleton that surround the soft tissues are often removed. Endoprostheses are placed in place of the removed bones.

But doctors try not to bring to such a radical treatment. First, other, less traumatic methods are used. For example, partial resection of the tumor is practiced. This improves the effectiveness of chemotherapy and the chances of recovery.

Radiation therapy

High doses of radiation are used for treatment - 4000-5000 R. The impact is made on the primary focus of the neoplasm, and if metastases have spread to the lung tissue, then to the lungs. Side effects of such treatment can be headaches, limited movement of the joints, in children - this is a slowdown in bone growth and impaired mental activity. But, despite this, radiotherapy is now the most effective and safe method of treating the disease.

Chemotherapy for Ewing's sarcoma

This treatment is effective because medications capable of destroying even the smallest metastases that cannot be diagnosed. The most common chemotherapy drugs are Cyclophosphamide, Adriamycin, Vincristine, Ifosfamide, Vepezid. They are prescribed most often intravenously, monthly courses with small - in 2-3 weeks - breaks. This treatment usually lasts 10-12 months. Often different drugs are combined with each other. Chemotherapy is used in the initial stages, as well as before and after surgery.

The use of such drugs has many side effects. Although modern medicines are much easier to carry, but still often observed:

• nausea, vomiting;
• hair loss;
• decreased immunity;
• infertility;
• toxic damage to the cardiovascular system.

In the presence of large metastases in the bone marrow, the prognosis of the disease is the most unfavorable - the survival of patients is only 25%. Therefore, they are used higher doses chemotherapy combined with stem cell transplantation.

The success of recovery depends not only on the correct treatment, but also on the psychological mood of the patient himself.

What does recovery depend on?

The success of treatment depends primarily on the patient himself. Despite the fact that Ewing's sarcoma is considered one of the most serious and dangerous diseases, it can be cured. You just need to contact a medical institution in time, follow all the recommendations of the doctor and believe in recovery. The psychological attitude is very important, as well as the support of loved ones. Now more than 70% of patients after recovery get a chance to normal life without relapses.

This disease develops very quickly: within a few months after the appearance of the first pain symptoms, the tumor becomes visible externally. In 90% of cases, metastases spread, most often to the bone marrow and lung tissue. And in the later stages, the tumor almost always metastasizes to the central nervous system. Therefore, it is very important to start treating the disease as early as possible.

Even after recovery, patients need dispensary observation in order to notice the development of a relapse of the disease in time and prevent complications. Most of the time it is for life. Only a small percentage of patients after recovery can return to a full life.

Ewing's sarcoma - very dangerous disease affecting children and adolescents. The further life and health of such patients depends on how the parents noticed the first symptoms of the disease in time, how they followed the doctor's recommendations, what efforts were made to recover. The disease is dangerous because it is impossible to prevent the development of this tumor, since the reasons for its appearance have not been fully investigated. But the current level of development of medicine makes it possible for 70% of patients to be completely cured.

It is a malignant neoplasm, mainly localized in hard tissues(joints, cartilage and bone tissue). Because bone sarcoma is oncological disease, but the nature of its occurrence is unknown.

Basically, the causes of the appearance of a malignant tumor in the bones are associated with the presence of cancers in other organs, which are formed in the most mobile parts of the body.

Causes of bone pathology

As a rule, allocate the following reasons appearance of a bone tumor

• action of viruses;
• increased growth of bone tissue;
• the action of hazardous chemical elements, carcinogens;
• fibrous dysplasia;
• Paget's disease;
• injury.

Symptoms

The main symptoms of bone tissue damage are associated with pain. The pains are deep, strong, growing, constant or aching. They do not depend on the position of the body or physical activity. As a rule, patients have severe pain at night, so they suffer from insomnia.

Other symptoms for bone sarcoma are as follows:

1. violation of normal functionality in the place where bone sarcoma and Ewing's disease develop;
2. swelling in the affected area, the appearance of a venous network on the limbs;
3. the presence of pathological fractures;
4. a sharp decrease in body weight;
5. insomnia;
6. fever and anemia;
7. rapid fatigue and weakness.

In the late stage, the symptoms of the disease are associated with acute pain, lameness, metastasis, and muscle atrophy. With sarcoma, the tumor is dense, attached to the bone and does not have clear boundaries. With the gradual progression of the disease, functional cells are destroyed, and bone fragility appears.

Characteristics of bone sarcomas

Sarcoma of the bone is of the following types:

• sarcoma femur;
• tibial injury;
• humerus injury;
• damage to the hip, frontal bone,;
• ewing's sarcoma.

A tumor of the femur can be primary or secondary, appear in the upper limb, or be a metastasis. With progression, the pathological process spreads to the knee or to the hip joint. Per a short time the process captures adjacent . When the cartilage of neighboring joints is involved, chondroosteosarcoma of the thigh develops.

Tibial sarcoma is popular among osteogenic neoplasms, develops from the bones of the limb and is localized near large joints (mainly near the bones of the thigh and tibia). In the affected area, the bone structure changes, the joints become brittle and fragile.

Now different bones are known (including Ewing), which differ in the level of negative effect on the bone. Before the treatment of pathology is started, the main symptoms of the disease are determined.

The defeat of the humerus refers to secondary lesions of the bone tissue, that is, it develops due to the sarcoma of the trunk. At the initial stage of growth, the tumor of the humerus does not have pronounced signs. It is located under the muscle tissue, where the gradual progression of the pathology occurs with further clamping and damage to blood vessels and nerves. When the shoulder sarcoma spreads upward to the nerve plexus, this area becomes painful and joint mobility is reduced.

With sarcoma of the frontal bone, the occipital and frontal zones skull, and intracranial pressure increases. Before the treatment of pathology is prescribed, an x-ray examination is performed.

Sarcoma of the pelvic bones is localized in the same way as Ewing's neoplasm. The initial symptoms of the pelvic bone tissue are associated with the appearance dull pains, increased body temperature. With the growth of the tumor, the organs are compressed, the skin becomes thinner, the near ones are squeezed. blood vessels.

Signs of bone cancer

Tumor of the ilium or extends to the flat bones. With difficulties in identifying sarcoma of the ilium on early stages, it is often detected during metastasis in, the treatment of which is impossible.

Regardless of the distribution of Ewing's sarcoma on tubular bones, the tumor can be detected even on flat bones (including the ilium).

In children, bone sarcoma is mainly represented by Ewing's tumor and. These are oncological lesions of bone tissue, which are formed from malignant cells. The lesion occurs mainly in the tubular bones, affecting the metaphysis. With Ewing's sarcoma, fat and muscle tissues, as well as tendons, can be affected.

Stages of development

This disease has 4 stages of development, each of which is characterized by individual signs and symptoms.

At the first stage, there may be no pain and discomfort due to the small size of the tumor; the second stage is characterized by pain in the bone tissue in the area of ​​the tumor.

For tumors at the 3rd stage of development, metastasis, increased pain and an increase in size are inherent. On the last step the disease is considered intractable and neglected, since metastases spread to many organs, due to the large size of the neoplasm, other organs are squeezed.

Diagnostics

Before the treatment of the pathology of the joints and bones is started, a diagnosis is made. It is performed on the basis of symptoms of pain, disorders of the organ. X-ray analysis, biopsy of the contents of the tumor, computed tomography, bone scintigraphy, MRI and blood tests are also performed.

Choice of treatments for bone sarcoma

As in the treatment of other sarcomas, methods are selected depending on the stages of tumor development. Treatment will be more effective in the earliest stages of the disease.

In the course of therapy, the following drugs: adriamycin, imidazole-carboxamide, cyclophosphamide.

radiographic picture knee joint with Ewing's sarcoma

Surgical treatment includes amputation of the injured limb or extriculation. An alternative to surgery is radiation therapy and the use of antitumor drugs.

Preventive measures and forecasting

Even when using modern treatment full regression of pathology cannot be guaranteed. That is, malignant tumors are difficult to treat, so it is better to reduce the risk of their occurrence. To preventive measures include the passage of a systematic examination, getting rid of bad habits, increase physical activity, lifestyle and dietary changes.

The prognosis of bone disease is established in accordance with an objective assessment various factors and general condition of the patient. The use of modern methods of chemotherapy, radiotherapy and surgical intervention improves the survival of patients.

Ewing's sarcoma is a malignant neoplasm that affects the skeleton, in particular the spine, pelvis, ribs, shoulder blades, collarbones and lower sections of long bones.

general information

Ewing's sarcoma was first described in 1921 by Dr. James Ewing. It is considered one of the most dangerous and aggressive cancerous tumors because it has a high tendency to metastasize. Most often, secondary foci are found in the lungs and bone marrow.

The most susceptible to the disease are children from 10 to 15 years old, related to caucasian race. The tumor is slightly more common in boys than in girls. Ewing's sarcoma in children in most cases affects the femur, shoulder and tibia. At an older age, the disease affects the ribs, vertebrae, shoulder blades, flat bones of the skull and pelvis. In 70% of cases, regardless of age, the tumor is localized in the lower extremities and bones of the pelvic girdle.

Round cell tumor of soft tissues has similar features with bone cancer. It was described in 1969 and given the name extraskeletal Ewing's sarcoma. These forms of cancer have common morphological and clinical manifestations, but differ in the place of localization: the classic Ewing's sarcoma affects the bones, and the extraskeletal one affects the soft tissues of the spine, chest, retroperitoneal space, and so on.

The reasons

The cause of Ewing's sarcoma has not been established. Scientists believe the disease is triggered genetic factors. This assumption is confirmed by the fact that a bone tumor is often found in siblings.

Genetic studies of neoplasm tissues show that in most of its cells there is a chromosomal translocation (mutation) and the expression of one of the proteins, which is normally detected in the neuroectodermal tissue during prenatal development.

Studies show that there is a relationship between the development of Ewing's sarcoma and bone injuries, as well as some pathologies, including:

• skeletal anomalies - enchondroma ( benign tumor in cartilage tissue), bone cyst (a cavity in the thickness of the bones);
• congenital defects of the genitourinary system - hypospadias (violation of the structure urethra in boys), reduplication (doubling) of the renal system.

Pathogenesis

What is Ewing's sarcoma disease? Until 1980, it was believed that bone cancer is of an endothelial nature, that is, it is formed from cells lining the surface of blood vessels and internal cavities. New research has shown that Ewing's sarcoma is formed from elements of nervous tissue.

In the structure of Ewing's sarcoma there are small round cells separated by fibrous layers. The cells are characterized by the correct shape, inside them are rounded or oval nuclei with chromatin and basophilic nucleoli. Ewing's sarcoma does not produce osteoid - new bone tissue.

The mechanism of bone tumor formation is uncontrolled clonal proliferation (division) of cells. Scientists believe that it is triggered by specific genetic damage, in particular a mutation of a gene that encodes the production of a receptor that transforms one of the growth factors. As a result, there is no suppression (suppression) of cell division with genetically modified information. This is how cancer develops.

Doctors distinguish two main stages of Ewing's sarcoma:

• localized - the tumor forms in the bone tissue and can affect nearby muscles and tendons;
• metastatic - cancer spreads throughout the body.

A feature of Ewing's sarcoma is that the progression of the disease occurs in a short time, especially in childhood.

Metastases are secondary foci of malignant neoplasms: cancer cells enter the blood and lymphatic vessels, are transferred to other organs, are fixed there and begin to grow, forming new tumors.

In Ewing's sarcoma, in many patients, metastases are found in the lungs, bone marrow, and bones during primary diagnosis. With the generalization of pathology, secondary foci are always detected in the central nervous system.

In addition to the specific classification, the traditional division into 4 stages is applied to bone cancer:

• the first is a tumor on the surface of the bone;
• the second - the neoplasm grows into the thickness of the bone;
• the third - metastases occur in nearby organs and tissues;
• fourth - distant foci are detected.

Stages 3 and 4 of Ewing's sarcoma refer to the metastatic period.

Symptoms

Symptoms of Ewing's sarcoma depend on the location of the tumor and the degree of tissue damage. At the first stage, there pain in the field of innovation. Their features:

• are unstable;
• increase with the progression of the disease;
• do not subside when fixing the limb;
• aggravated during rest, especially at night.

Due to the growth of Ewing's sarcoma, it disrupts the nearby joint up to its complete immobilization. Most often this happens 3-4 months after initial symptoms. During the same period, local changes may be detected:

• redness and swelling of tissues;
• the appearance of a tumor formation;
• pain on palpation;
• visually noticeable expansion of veins and capillaries;
• skin hyperthermia.

In addition, regional lymphadenitis (enlargement of adjacent lymph nodes) is observed, and bone fragility increases, due to which fractures occur even with minor injuries.

In addition to specific manifestations, Ewing's sarcoma is accompanied by common symptoms, among which:

• weakness and fatigue;
• loss of appetite and weight;
• anemia;
• hyperthermia - up to 38 ° C.

Signs of systemic intoxication are among the first to occur, but in most cases they are mistaken for manifestations of another disease.

Symptoms of bone cancer have some features depending on the location of the lesion:

• lower extremities and pelvis (for example, Ewing's sarcoma of the ilium) - lameness;
• vertebrae - radiculopathy, spinal cord injury, muscle weakness, slow reflexes, paralysis;
• chest - respiratory failure, blood with sputum.

Diagnostics

The basic method for detecting Ewing's sarcoma is radiography: it makes it possible to visualize the tumor, determine its size and stage of development. Features of the X-ray picture in the study of bones affected by Ewing's sarcoma:

• a combination of osteosclerotic and destructive processes of bone formation;
• stratification, fuzziness of contours and division into fibers of the cortical layer;
• small needle and lamellar formations in the structure of the periosteum;
• change in soft tissues near the tumor, which often exceeds it in size;
• the absence of cartilage, calcifications and elements of pathological bone tissue in the soft tissue component.

In addition, when examining a patient with suspected Ewing's sarcoma, such instrumental methods are used as:

• computed and magnetic resonance imaging - allow you to accurately assess the size of the neoplasm and its connection with the neurovascular bundle, as well as the presence of metastases;
• x-ray and CT of the lungs - show secondary cancerous foci;
• positron emission tomography (PET), bone scintigraphy, angiography, ultrasound are used to detect distant metastases.

To clarify the histological type of the tumor, a biopsy is performed - a small sample is taken from the affected area and / or soft tissue component. During the analysis, cells typical of Ewing's sarcoma, necrosis of the tumor tissue and the concentration of viable cells near the vessels are detected. A bone marrow biopsy from the iliac bones is also mandatory.

During the diagnosis, bone cancer is differentiated from neuroblastoma, non-Hodgkin's lymphoma, rhabdomyosarcoma and other types of malignant tumors. A biopsy of a neoplasm does not make it possible to reliably determine the type of cancer, therefore, an additional molecular genetic study is carried out, including:

• fluorescent hybridization - shows a chromosomal translocation - a pathognomonic symptom of Ewing's sarcoma;
• polymerase chain reaction - allows you to detect micrometastases in the blood and bone marrow;
• immunological study - makes it possible to identify a glycoprotein synthesized by bone tumor cells.

Blood tests show leukocytosis, increase in ESR and increased levels of certain enzymes.

Treatment

Treatment of Ewing's sarcoma involves the impact not only on the primary focus, but on the whole organism as a whole, even in the absence of metastases. This approach is justified by the high aggressiveness of bone cancer and the likelihood of undetected micrometastases. Therapy is carried out by conservative and surgical methods.

Components conservative treatment Ewing's sarcomas - chemotherapy and radiation therapy. They are practiced before and after surgery, as well as in inoperable cases.

Chemotherapy involves the use of several drugs in an individually selected combination. Drug combinations are changed every 3-4 weeks. The cycles are repeated 4-5 times. The effect of chemotherapy is considered positive if less than 5% of viable tumor cells are found after the course of treatment. With Ewing's sarcoma, vincristine, cyclophosphamide, phosphamide, etoposide, adriamycin, vepezid and other drugs are prescribed.

Side effects from chemotherapy:

• nausea, vomiting;
• hair loss;
• violation of reproductive functions;
• heart disease;
• a sharp decrease in immunity and so on.

Radiation therapy - external ionizing radiation in high doses (up to 45-55 grays). The primary focus and lungs are usually treated (in the presence of metastases in them). Ewing's sarcoma is highly sensitive to radiation, but this technique has a number of side effects, including:

• violation of bone growth;
• defeat Bladder and intestines;
• joint deformity, etc.

The choice of the type of surgical intervention is carried out depending on the location and depth of the lesion. Previously, with Ewing's sarcoma, in most cases, operations were performed to remove organs. Today, safe interventions are practiced: a resection of the affected bone is performed, and a prosthesis is installed instead. Such operations are successfully performed on the peroneal, radial and ulna, as well as on the clavicles, ribs and shoulder blades. At certain conditions surgeons manage to replace the bone graft of the pelvis, hip and shoulder.

If it is impossible complete resection tumor is carried out partial removal. This increases the level of control over its development.

New treatments for Ewing's sarcoma are based on bone marrow and peripheral stem cell transplantation. Such an operation increases the chances of recovery in patients with bone marrow damage.

Forecast and prevention

The survival prognosis for Young's sarcoma depends on the stage at which treatment was started, the location of the tumor, and the presence and location of metastases. With a localized bone tumor, removed before the appearance of secondary lesions, the survival rate is 70%.

The terminal period of the disease, accompanied by multiple metastases, has an unfavorable prognosis. For example, stage 4 Ewing's sarcoma of the ilium is associated with a very high mortality rate of about 90%. But subject to the use of high doses of chemotherapy and radiotherapy, as well as in the case of bone marrow transplantation, the survival rate increases to 30%.

After successful treatment, patients should be regularly examined by an oncologist in order to detect relapses of Ewing's sarcoma in time. In addition, many people need ongoing drug therapy due to side effects. anticancer drugs and irradiation.

Prevention of Ewing's sarcoma is impossible, since the causes of the disease have not been reliably established.

Ewing's sarcoma is a disease caused by a malignant tumor of the bone skeleton and affects all skeletal bones, but more often long tubular bones in the lower part, pelvic bones, spine, shoulder blade, ribs and collarbone. After reading the article, you will learn about the causes of Ewing's sarcoma, its classification by location, as well as methods of diagnosis and treatment.

In 70% of cases, Ewing's sarcoma is diagnosed in the lower extremities, especially in (17-27%), pelvis (21-25%). A formation is found in the fibula - 11%, in the tibia - 11-15%, in the ribs - 10%, in upper limbs- up to 16% of cases.

Most often it occurs in adolescents aged 10-15 years (in 10-15% of cases), less often in children under 5 years old and guys after 25-30 years old. In boys, the disease is detected 1.5 times more often than in girls. With active growth of the skeleton and during puberty, the risk of the disease increases.

malignant tumor bone skeleton

Oncological formation refers to round small cell tumors with varying degrees of neuroectodermal differentiation. PAS - positive cells are spindle-shaped, so it is required differential diagnosis before prescribing treatment. This is necessary because of some connection between skeletal anomalies (enchondroma, aneurysmal bone cyst, etc.), in the occurrence and development of sarcoma, and genitourinary anomalies(hypospadias, reduplication of the renal system).

There are several subtypes of Ewing's tumors: and atypical Ewing's sarcoma, PNET or MPNET - neuroectodermal peripheral malignant tumors (occur in 15% of cases). Ewing's malignant tumor is characterized by aggressiveness, early metastasis to the bone marrow, adjacent soft tissues, kidneys or liver. In 20% of patients, multiple micrometastases occur due to lymphogenous spread. CNS metastases account for 2.2% of cases.

When diagnosing Ewing's disease, the main marker CD99 or (serum neuron-specific enolase) is used. The tumor can also be expressed with Vimentin.

Causes of Ewing's sarcoma

The exact causes of Ewing's sarcoma have not been determined, we can only talk about the suggestive factors influencing the occurrence of the oncological process.

So, you can get sick because of:

• precancerous benign bone tumors;
• genetic predisposition or inherited disorders;
• chromosomal changes that disrupt certain genes;
• bone traumatism, with a short or long time interval between the injury and the development of education;
• anomalies in the development of the bone tissue of the skeleton (bone cysts, enchondromas and others);
• intrauterine development disorders.

Classification: types, types and forms of Ewing's sarcoma

Ewing's sarcoma code according to ICD-10:

1. C40. malignant neoplasm bones and articular cartilage limbs.
2. C41. Malignant neoplasm of bones and articular cartilage of other and unspecified localizations.

Ewing tumors include the following types:

1. atypical Ewing's sarcoma;
2. Askin's tumor (thoracopulmonary region);
3. malignant peripheral neuroectodermal tumor of bones and soft tissues. Peripheral primitive neuroectodermal tumor (PNET Ewing's sarcoma), with the localization of the primary focus in soft tissues, accounts for 15% of cases.

Sometimes they spend differential diagnosis between PNET (primitive neuroectodermal tumor) and Ewing's sarcoma. Although anatomically unrelated to the structure of the CNS or the autonomous systemic system of nerves, its neuroectodermal nature is evidenced by cytogenetic changes. In tumor cells, the expression of the PAC3 protein is detected during the embryonic development of the neuroectodermal tissue.

Classification of Ewing's sarcoma by location:

Above the site of localization of oncological formation, the tissue changes, swells, becomes red and hot. The vascular pattern increases and the veins expand. When located near the joint inflammatory process can make movement difficult. Palpation causes pain. With soft tissue sarcoma of the sternum (Askin's tumor), effusion occurs in the pleural cavity, respiratory failure and hemoptysis occur.

• Ewing's sarcoma of the femur and knee

The disease is also called "osteogenic Ewing's sarcoma", it can be primary or secondary. The primary tumor originates in the upper leg area, grows aggressively and metastasizes early. The secondary tumor grows from metastases of the soft tissue of the thigh, genitourinary organs, sacrococcygeal zone. With progress, the pathological process spreads to the hip composition or to the knee, involving soft tissues.

The tumor looks like a bone sarcomatous node. It spreads along the length of the bone or develops along the periossal type on the surface of the bone, without penetrating into the medullary canal. If the cartilages of nearby joints are damaged, then femoral chondrosoteosarcoma occurs, and the risk of hip fracture increases. Pain occurs when the nerves are compressed and are felt throughout the lower limb, including the foot and fingers.

The oncological process in the knee occurs to a greater extent as a result of metastasis from other organs. Most often, older men suffer from knee sarcoma.

• pelvic bone

Cancer begins in the bone tissue, which is why the disease is also called osteosarcoma. If the growth of the node began in the cartilage tissue, then it is called. Pathological process leads to limited mobility and painful contractures at night and during palpation. Extremely aggressive development captures various organs in the oncological process due to early metastasis.

• Humerus (hand)

Oncological lesions often refer to the metastatic stage, that is, it is secondary.

The malignant process may initially be captured muscle, then as a result of periosseous spread over the surface of the bone:

1. bone tissue is destroyed;
2. other tissues and organs are affected;
3. the blood vessels and nerves of the shoulder are pinched and damaged;
4. pain and burning appear when the process spreads to the plexus of the nerves of the shoulder, which responds to the innervation of the entire limb up to the nails;
5. sensitivity is disturbed (the hand “goes numb”, “goosebumps” appear);
6. motor functions are disturbed: muscle tone decreases, the hand weakens;
7. the motor skills of the fingers deteriorate, it is difficult to hold objects in the hand and manipulate them;
8. the skeletal system weakens, shoulder fractures are possible.

The primary oncological process spreads to the hands from tumors of the head, neck, sternum, and spine. From the bone tissue of the shoulder, tumors metastasize to the lymph nodes: under the tongue, jaw, collarbone and armpits.

• Ribs and shoulder blades

In the early stages, there are no signs of cancer. Systematic pains under the ribs and shoulder blades begin with an increase in temperature, especially in the evening and at night. If the tumor mass grows from the bone outward, then the growth becomes dense and visible through the thinned red and hot skin. On the final stages pains become strong and sharp, they cannot be removed even by potent means. At the same time, metastasis is activated, the cells are taken up by the bloodstream and lymph. Patients are tormented by fever against the background high temperature up to 40 ° C, anemia increases.

• Jaws: upper and lower

It occurs almost always in children. The male population of 40-60 years old is at risk of the disease, it is rare in women. Lower jaw is affected by cancer more often than the upper one. Wherein:

1. tissue hypertrophy;
2. loosened teeth;
3. the temperature rises to 40°C;
4. there is pain and intoxication of the body,
5. cachexia (exhaustion) occurs;
6. the skin and mucous membranes become red and hot;
7. pus and mucus are released from the sinuses;
8. numb lips, jaws - "reduces";
9. ulcers form in the mouth;
10. a putrid odor spreads from the oral cavity;
11. the shape of the face changes and the cheeks swell.

Early metastasis is typical. The radiograph reflects the destruction of the jawbone.

• Spine and skull

Tumors cranial bone may be primary or secondary. Metastases even spread from the uterus or prostate gland, long tubular bones. When the brain is squeezed by a skull bone or a fixed hard tissue seal, headache and other complications. The tumor is prone to penetration into, characterized by early metastasis.

On the spine, pathological progress occurs rapidly due to active growth cancer cells. Patients suffer from movement disorders ranging from cervical spine to tailbone. Involved in the cancer process urinary organs small pelvis, their function is disturbed.

• Frontal bone and occiput

It occurs in young and middle-aged people. Education has wide base and a flattened spherical or ovoid shape, sometimes limited and consists of a spongy or compact bone substance. It grows continuously and can be invasive and endovasia forms.

A tumor of an invasive form can grow in the skull bones or inside its cavity, which is manifested by an increase in intracranial pressure, pain and other focal phenomena. A neoplasm can be examined on an x-ray, determine the direction of growth: inside the cavity or in frontal sinuses. Education requires immediate excision, which can be very difficult to carry out.

With an endovascular direction of growth, finding a formation on the outer cranial surface, even large operations are less difficult.

• Ilium

The pelvis consists of a combination of the iliac, pubic and ischium. They formed a bone ring with a bone wedge at the back from the bones of the sacrum and coccyx. In each of the bones, an oncological process can form in children and adults, since Ewing's sarcoma occurs not only in tubular, but also in flat bones. The disease is difficult to diagnose on x-rays, so an open biopsy is performed.

Stage classification according to AJCC/UICC

T - primary tumor:

• T - cannot be determined;
• TX - can be determined;
• TO - no signs;
• T1 - the cortical layer does not germinate;
• T2 - infiltrates on one side and grows into the periosteum.

N - Regional lymph nodes:

• NO - there are no metastases in regional lymph nodes;
• Nl - metastases in regional lymph nodes were determined.

M - Distant metastases:

• MO - distant metastases are absent;
• M1a - lung metastases detected;
• M1b - other distant metastases were found.

pTNM: pathological classification

G: histopathological gradation

• G1 - high degree of differentiation.
• G2- average degree differentiation.
  G3 - undifferentiated.
• G4 - undifferentiated.

Ewing's osseous sarcoma is always classified as G IV.

Stages of the oncological process

In a simplified version, today there are two stages of the disease:

• localized, with one focus of the tumor in the bone tissue, spreading to the muscles and tendons located nearby;
• metastatic, with the appearance of foci in other organs.

At a localized stage, the following types of tumor are distinguished:

• oncoprocess in the outer shell of the bone;
• cancer process in the external and internal parts;
• cancer process in the surrounding tissues.

The metastatic stage is divided into large metastatic type and small metastatic type.

For diagnosis, advanced stages of Ewing's sarcoma are used:

• 1 stage. A small knot in the bone tissue.
• 2 stage. The tumor grows deep into the bone.
• 3 stage. Metastases in nearby tissues and organs;
• 4 stage. distant metastases.

Ewing's sarcoma: symptoms and manifestation

Common symptoms of Ewing's sarcoma are:

• moderate and acute pain syndrome;
• sleep and performance disorders;
• fever, fatigue and irritability;
• limitation of joint mobility;
• bone fractures;
• an increase in the size of formations, skin temperature above them;
• swelling of soft tissues, dilatation of the saphenous veins;
• general weakness, anemia, loss of appetite and weight;
• regional lymphadenitis: an increase in the nearest lymph nodes;
• local symptoms: on the leg - lameness, on the vertebrae - compression-ischemic myelopathy, destruction of the vertebrae, on the ribs and shoulder blade - respiratory failure, hemoptysis,

Diagnostics

Diagnosis of Ewing's sarcoma is carried out by a group of doctors: an oncologist, a traumatologist, an orthopedist and a surgeon.

In a clinical study, the diagnosis of Ewing's sarcoma is confirmed by:

• initial examination and palpation;
• x-ray, CT and MRI;
• positron emission tomography (PET);
• biopsy, including penetrating needle and open;
• radioisotope method;
• morphological diagnostics to determine the type of tumor;
• molecular biology and immunohistochemical methods;
• combined PET + CT.

CT / MRI is performed to clarify the location and size of the tumor, identify the degree of soft tissue damage, assess the condition medullary canal and detection of metastases. The connection of the onconode with the neurovascular bundle is determined. Rg-graphy/CT of the chest reveals tumor metastases.

Biopsy reveals small blue malignant monomorphic cells o round shape With varying degrees neutral differentiation. CD99 is found in the biopsy material. At cytological examination determine the rearrangement of chromosome 22, which indicates oncology.

Ultrasound shows an increase in peripheral lymph nodes, in the organs of the peritoneum - destructive changes and hepatosplenomegaly. In the pelvic organs - free fluid. Ultrasound also examines the retroperitoneal space, pleural cavities, soft tissues to reveal the prevalence of the oncological process.

An electrocardiogram is performed to determine the signs of cardiomyopathy, a myelogram is performed to detect pathological cells in the bone marrow. The hemogram shows the level of leukocytes, erythrocytes, platelets, hemoglobin and leukocyte formula. This test is necessary in order to identify absolute contraindications to chemotherapy, to monitor the patient's condition during special therapy, taking into account its toxicity.

According to indications, the level of oncomarkers, metabolites of catecholamines in the urine, lactate dehydrogenase in the blood is determined. Identification of affected vessels doppler ultrasound. Before starting treatment, height, weight and BSA - body surface area are measured. Menstrual cycles are recorded in women and pregnancy testing is carried out. Determine lung metastases by testing the functional work of the lungs.

The diagnosis is confirmed radiological signs.

On the x-ray in two projections, you can see:

1. destruction of bone tissue;
2. penetration of tumor masses into soft tissues;
3. reactive bone formation;
4. thickening of the periosteum, sclerosis and fractures.

In the laboratory, a general analysis of urine and blood is examined, biochemical analysis blood to determine the total protein and C - reactive, alkaline phosphatase, LDH, liver enzymes, dysmetabolic changes (potassium, calcium and sodium levels). BAK determines hypoalbuminemia, hypoproteinemia, increased transaminases.

According to the detailed CBC (general blood test), the presence of leukocytosis / leukopenia, anemia, thrombocytopenia (if metastases in the bone marrow are detected) is determined, increased ESR. According to the coagulogram - the presence of discoagulation changes. OAM reveals urate/oxalaturia.

Differential diagnosis is carried out to exclude:

• acute hematogenous osteomyelitis;
• osteosarcoma and other bone tumors;
• metastatic (secondary) bone lesions;
• bone tuberculosis.

If necessary, provide emergency assistance:

• complaints, anamnesis of illness and life are taken into account;
• the status is objectively assessed: how the affected limb is located, the mobility of the adjacent joint, swelling, local hyperemia, the presence of pain at the site of the lesion are detected;
• body temperature and blood pressure are measured;
• examine the radiological signs of Ewing's sarcoma (the presence of an oncological process and a fracture);
• prescribed drug analgesic therapy, antipyretics.

Treatment of Ewing's sarcoma

With a confirmed diagnosis of "Ewing's sarcoma", treatment is prescribed non-drug and drug.

Non-drug treatment

It is necessary to adhere to sparing protective regime, excluding injury, hypothermia and overheating, active sports, contact with infectious patients and large crowds of people. You can not take hot baths, go to baths and insolation. Water procedures at a water temperature of 33-36 ° C should last up to 10-15 minutes.

In the presence of blood agranulocytosis with Ewing's sarcoma, the oral cavity should be treated with antiseptics, and teeth should be cared for with foam brushes. If a central catheter is present, it should be Teflon. It is forbidden to change it along the line. Hands should be properly treated, personal hygiene should be observed. Gloves, masks and syringes should always be available. Patient rooms should be isolated, with laminar air flow.

Treatment of Ewing's sarcoma is carried out in compliance with an individual diet:

1. table number 11 according to Pevzner: increased energy intensity is achieved by an increase in dairy and protein products, the introduction of vitamin-mineral complexes into the diet;
2. with low-bacterial food: table No. 11, No. 1B, No. 5P, No. 16 (for children under 3 years old), No. 16 when feeding through a probe;
3. with an increased amount of calories (1.5 times more protein), vitamins, minerals. When treating with glucocorticoids, the diet is enriched with products with potassium and calcium salts.

The diet should be 5-6 times a day and in small portions. There is no restriction on the processing of products. With PCT, fried foods with hot spices and salt are excluded. If myeloablative cytopenia worries after chemotherapy, exclude from the diet fresh vegetables and fruits to exclude intestinal enteropathy. At the same time, a high-calorie diet is prescribed, but spicy, fatty, fried protein foods are excluded.

Medical treatment

If a fracture is suspected, an x-ray is performed and the affected limb is immobilized, anesthetized and hospitalized.

When conducting antibiotic therapy to exclude pneumonia after a course of PCT, treatment is carried out:

1. trimethoprim sulfamethoxazole;
2. Amoxicillin and clavulonic acid (if the level of leukocytes in the blood has decreased and is 2x109 / l and below).

Antifungal therapy is carried out: Nystatin and Phenol. Antiviral therapy - Acyclovir. Anesthetize with Metamizole sodium and Ketonal.

Colony-stimulating therapy (with low leukopenia - 1x109 / l) is performed in a day hospital with Filgrastim. Metoclopromide is prescribed for vomiting. With nosebleeds, an anterior tamponade is placed and treated with sodium etamsylate.

Medication treatment for standard risk R1, high risk R2, and very high risk R3

Patients who have a good R1 response (viable tumor cells make up< 10%) и опухоль отграниченна, получат лечение химиотерапией из 8 циклов. Для больных женщин применяется лечение , Актиномицином D и Циклофосфамидом (VAC), для мужчин - вместо (VAI). При необходимости назначают Фенретинид либо Золедроновую кислоту, либо оба препарата.

If the response is poor (R2) and the tumor is localized, 8-cycle VAI chemo or high-dose chemo with Busulfan-Melphalan (R2loc) will be administered. For lung metastases, 8-cycle VAI chemo or high-dose chemo Busulfan-Melphalan (R2pulm) is also given.

If at R3 the lesion is widespread, there is dissemination in the bone and / or in other areas, the lungs are affected, then patients are prescribed VIDE induction chemotherapy of 6 cycles. Patients are randomized to receive 8 cycles of treatment with Vincristine, Actinomycin D and Cyclophosphamide (VAC) or high-dose chemotherapy with Thiosulphate-Melphalan (TreoMel). Next, autologous reinfusion of stem cells is performed, followed by 8 cycles of VAC chemistry. Topical therapy in patients at risk of R3 is performed following VIDE induction, prior to high-dose treatment.

After high-dose treatment, surgical operation, for example, for the purpose of reconstructing the pelvic bones, since the immobilization period will be long. Prior to high-dose chemotherapy and surgery, radiation therapy is carried out, if necessary.

Chemotherapy for Ewing's sarcoma is considered the main type of treatment. Upon receipt of 6 cycles of VIDE chemo, if possible, the tumor is removed, then pathological area irradiated.

• MESNA 1.0 g/m2/day - 1 day in / in the stream before the introduction of Ifosfamide.
• MESNA 3.0 g/m2/day – 1.2.3 days of IV infusion, 24 hours.

Informative video

Radiation therapy

Irradiation is carried out when it is impossible to perform the operation. Preoperative radiation therapy is performed simultaneously with the 5th and 6th or even with the 3rd and 4th chemotherapy blocks in critical situations: with spinal cord compression or tumor progression during chemotherapy. For large tumors with a central axial location, irradiation may be postponed for the duration of treatment with Busulfan and. These drugs are toxic and should not be used after exposure.

Radiation therapy for Ewing's sarcoma in some patients begins after chemotherapy, also performed after surgery. Time is given for the patient to recover. Spend VII blocks of chemistry, then irradiation. In patients with residual tumor, radiation therapy is started earlier.

After high-dose chemotherapy with Busulfan and Melphalan, the interval between stem cell reinfusion and radiation therapy is 8-10 weeks, this is necessary to exclude rejection.

Before surgery, irradiation is carried out at a total dose of 54.4 Gy. In order to carry out preoperative chemotherapy for Ewing's sarcoma in full, preoperative radiation is prescribed individually according to the accelerated split (hyper fractionated) course.

In irradiation, remote 3-D and intensively modulated (IMRT) radiation therapy is also used and combined with chemistry, in case of failure or non-radical surgery performed as a treatment. Doses: ROD 2-2.5 Gy, SOD - 60 Gy. In the presence of metastases in the lungs, doses of large-field radiation therapy - ROD - 2 Gy, SOD - 20 Gy.

When dosing postoperative irradiation based on the concept of "surgical edge". Namely:

1. with partial cutoff of the formation of SOD = 54.4 Gy;
2. with marginal clipping + poor histological response (there are viable tumor cells in in large numbers) - SOD = 54.4 Gy;
3. with marginal cut-off + good () histological response (viable cancer cells decreased to 10%) - SOD = 44.8 Gy;
4. with a wide excision + poor (less than 10%) histological response SOD = 44.8 Gy.

Surgical treatment

As with radiation therapy, radical surgical treatment provides local control over the oncological process. Choose the scope of the operation individually. It depends on how the primary focus is located, what size it is and the response to the chemotherapy. The age of the patient and the expected prognosis are taken into account.

In onco-orthopedics, surgical treatment is divided into types:

1. mutilating operations (amputation of limbs or parts thereof is performed);
2. organ-preserving operations:

• subsequent reconstruction of the defect is not carried out;
• the defect is reconstructed.

The main condition for the operation is the radical and ablastic removal of the bone tumor, that is, all cancer cells are removed in order to prevent local recurrence.

Radical operations are also divided into types:

• amputation or disarticulation of limbs (complete or partial exarticulation along the joint space, bone filing is not performed).
• bone resection, with a minimum required indentation from the edge of oncology, equal to 3-5 cm, if there are no tumor cells in the brain of the left bone.
• the node is removed without opening the pseudocapsule.

Cripple operations are prescribed in the presence of:

• extensive primary-multiple spread of cancer formation;
• a significant extraosseous component of the tumor involved in the oncological process along the main neurovascular bundle. It excludes the reconstructive-plastic stage of the operation;
• negative effect of conservative therapy;
• refusal of the patient from surgery with preservation of the organ;
• disintegrating tumor and bleeding.

In organ-preserving operations, endoprostheses are used, which improves the quality of life of people. They are placed even in rare anatomical zones.

Prostheses must be:

• modular and versatile;
• the most reliable and maintainable;
• with stable fixation;
• with maximum service life;
• biologically inert to the structure of bones and soft tissues, have a special protective layer on the metal to prevent poisoning of the body;
• With short term manufacturing.

In children, prostheses should be fixed without cement, have an invasive and non-invasive sliding mechanism, due to the active regenerative capabilities of the bones. With the growth of bones, the mechanism will allow for a second revision surgery and, if necessary, to replace part of the endoprosthesis.

If the patient has a significant limb deficiency and there is no possibility to immediately perform intraoperative correction due to osteolysis or bone fracture, an expandable module is installed and the limb length is gradually adjusted.

homeopathic treatment

In addition to other methods of treatment, immuno-enhancing and antitumor natural homeopathic preparations are used for Ewing's sarcoma.

You can increase immunity with alcohol tinctures (20 drops per day before meals):

• ginseng root;
• roots and leaves of Eleutherococcus senticosus;
• roots of Aralia Manchurian (40 drops, 3 times);
• leaves and rhizomes of Rhodiola rosea;
• roots and herbs echinacea.

Echinacea can be used to:

1. infusion: grind the herb (100 g), steam 200-300 ml of boiling water and insist on the bath for another 15-30 minutes. Drink ¼ st. before meals - 3 times;
2. decoction: chop the roots (300 g), steam with boiling water and simmer in a bath for up to 50 minutes, separate the thick and drink 1 tbsp. l. three times a day.

It is useful to prepare and take extracts from Altai herbs: licorice, wild rose, St. John's wort, meadow geranium, kopek and others.

Fees are made up of the most popular healing medicinal herbs(in parts by weight):

• wormwood (3);
• licorice root (3);
• motherwort (2);
• dandelion root (2);
• St. John's wort and lungwort (2);
• mint and rose hips (2);
• raspberry and birch leaves (6).

To prepare the infusion, steam 1 tbsp. l. collection with boiling water (500 ml) and insist 60 minutes. Drink before meals ¼ tbsp.

Immunoboosting drinks for children are prepared as follows:

• an aloe leaf is cut and mixed with homemade milk (100 ml), honey (3 tablespoons), lemon juice and walnuts (4-5). Insist 48 hours and give the child 1 tsp. before meals;
• an infusion is prepared from the flowers of black elderberry (1 tbsp.), Steamed with boiling water (1 tbsp.). They let it brew, filter and drink 0.5 tbsp before going to bed;
• from walnut leaves (2 tablespoons) prepare an infusion in boiling water (500 ml), insist 12 hours, filter and let drink 0.5 tbsp. twice a day.

From propolis tincture, birch buds and decoction oak bark make compresses and lotions. Ewing's sarcoma with metastatic lesions of the mammary gland is treated with herbs: cuff, astragalus, cinquefoil, serpentine root, oregano, St. John's wort, sage.

With a metastatic tumor in the lungs, they are treated with Spanish moss, lungwort, initial letter or cuff, motherwort or chicory root, horsetail and nettle, calendula and lingonberry, winter-loving, knotweed, succession and mistletoe, comfrey and cinquefoil (grass or root), sage or oregano, primrose, angelica, coltsfoot or elecampane leaf.

Useful turpentine baths: yellow at a temperature of 39 ° C. When exposed to hyperthermic baths, Ewing's sarcoma cells die, weaken and stop dividing, which stops the growth of oncology.

Consequences: metastases and recurrence

Therapy for metastases of Ewing's sarcoma or local relapses is palliative (temporary, with the use of half measures). In case of relapses, chemotherapy standards are not established, treatment is carried out with Cyclophosphamide, Ifosfamide, and drugs are combined with Etoposide and Topotecan (topoisomerase inhibitors). do not apply, since a cumulative dose of 550 mg / m² can be introduced earlier.

In metastases, standardized chemistry is used. In the presence of lung metastases, residual metastases are resected after chemotherapy and total lung irradiation. With metastases and enlarged mediastinal / hilar lymph nodes in the lungs, a surgical biopsy is also performed or foci are excised after induction chemistry.

Ewing's sarcoma: prognosis

In the presence of disappointing forecast, bone and bone marrow metastases, the 5-year survival rate is less than 10% of patients. In this case, mega-dosed chemotherapy, total body irradiation and transplantation of peripheral stem cells or autologous bone marrow began to be used. As a result, 30% of those with a widespread oncological process with metastases to the bones and their brain are cured.

If the chemotherapy performed on the tumor showed good sensitivity, then the remission of Ewing's sarcoma can be 7 years in 50% of patients. Excision improves local control of the tumor. When a complete resection is combined with chemotherapy and radiation, local recurrence is significantly reduced in frequency than after non-radical operations.

With localized Ewing's sarcoma, the survival rate for 5 years after adequate and timely combined treatment is up to 70% in the absence of metastasis.

The five-year prognosis for Ewing's sarcoma with metastases is only 20%. At early diagnosis, carrying out chemistry and radiation increase the prognosis in children with Ewing's sarcoma up to 60-70%. With metastasis to the bone marrow and irradiation - up to 30%. With metastases to the lungs and lymph nodes and the impossibility of excision, the prognosis is unfavorable.

After treatment and with a favorable prognosis, complications are possible. A person can suffer from cardiomyopathy, infertility, secondary oncological formations.

Prevention of Ewing's sarcoma

There is no definitive prophylaxis for Ewing's sarcoma. After the completion of the treatment program, patients are registered at the dispensary and undergo periodic examinations. They help to identify complications or recurrence, secondary development sarcomas.

Informative video

Oncological diseases are considered today one of the most dangerous and at the same time common ailments. modern medicine offers several treatment options pathological conditions but, unfortunately, not all of them are effective. As practice shows, many cancer-type diseases are diagnosed too late, since at an early stage of development they practically do not give any negative symptoms. In the event that the disease has gone too far, it is almost impossible to cope with it. One of the possible oncological diseases is Ewing's sarcoma. Let's talk about the features that are characteristic of such a disease as Ewing's sarcoma, consider 4 degrees of its development, causes, symptoms, treatment in a little more detail.

Ewing's sarcoma is oncological disease bone skeleton. Most often, doctors fix lesions of the tubular bones, the ribs, collarbone, scapular bones and spine suffer a little less often.

Why does Ewing's sarcoma occur? Causes of the disease

To date, doctors cannot accurately determine the factors that provoke the formation of Ewing's sarcoma. Many experts are confident in the genetic nature of this disease. As practice shows, the disease is often provoked by injuries that can be obtained long before the onset of the first symptoms of the tumor.

In addition to potentially hazards include the presence in the patient of benign formations of bone tissue, anomalies and disorders of the genitourinary system, as well as anomalies of the skeletal system.

How does Ewing's sarcoma present? Symptoms of the disease

This disease is extremely aggressive. It develops very quickly, and in the early stages it has unexpressed symptoms.

The classic manifestations of Ewing's sarcoma are considered to be deformation of bone tissues in the area tumor lesion, as well as the occurrence pain. At the same time, the pain does not decrease at all after fixation, only intensifying along with the progression of the pathology. Often the discomfort becomes more intense at night.

many consider initial manifestations ailment, like joint inflammation, however, in this case, not a single medication can cope with pain and swelling. Over time, bone deformities only increase, resulting in a violation of the functionality of the affected limbs. In the event that the sarcoma has formed on flat bones, for example, on the ribs, it makes the patient’s general motor activity an order of magnitude more difficult.

With an increase in the swelling zone, the patient may experience significant redness, an increase in local temperature, and pain during palpation. In addition, venous patterns often appear in these areas. Some patients experience pathological fractures bones.

If the sarcoma occurs on the leg, the patient begins to limp, its formation on the chest leads to pain during breathing and shortness of breath.

With the localization of the tumor on the spinal column, the patient may develop functional disorders in activity pelvic organs, paresis of the limbs, sensory failures or impaired motor functions.

In addition, the patient is concerned about the classic symptoms of cancer - weakness, decreased efficiency and appetite, weight loss, swollen lymph nodes, anemia, incomprehensible fever, etc.

How does Ewing's sarcoma progress? 4 stages

Most often, doctors talk about the four stages of development of Ewing's sarcoma.
1. At the first stage, the tumor is a small formation on the surface of the bone.
2. On the second - it grows inside the bone tissue.
3. The third stage is determined by the beginning of metastasis, while metastases are located in the nearest organs and tissues.
4. At the fourth stage, the tumor formation gives metastases to distant parts of the body.

How is Ewing's sarcoma corrected? Treatment of the disease

Since the disease is characterized by extreme aggressiveness, it must be treated systematically and quickly. Doctors are forced to expose the entire body of the patient therapeutic effect, because the probability of the spread of cancer cells through the blood is extremely high. For this purpose, a decision is always made to conduct polychemotherapy. Further, the patient also has to undergo radiation and prompt elimination primary site of injury.

Surgery it is advisable if the tumor is located on the extremities or flat bones. In this case, the removal of a bone fragment is carried out, which is then replaced with a special artificially created endoprosthesis. It is extremely rare that doctors are forced to amputate the affected limb.

After the surgical correction, patients are almost always prescribed radiation, since the cells of Ewing's sarcoma are distinguished by good sensitivity to radiation exposure.

Chemotherapy is usually given both before and after surgery. In this case, the preparations are selected depending on the results obtained during the study of pathological formation. In most cases, the drugs of choice are Etoposide, Ifosfamide, Doxorucbicin, Cyclophosphamide, and Vinkistin. The duration of chemotherapy exposure is approximately six months.

How dangerous is Ewing's sarcoma? Forecasts

In the event that treatment was started at the first two stages of the disease, then more than 70% of patients with such a diagnosis recover. In the presence of metastases, the number of survivors is significantly reduced - up to 20%.