Ewing sarcoma: symptoms of a tumor in children, treatment

Ewing's sarcoma in children: prognosis, stages, symptoms and treatment

Ewing's sarcoma - a malignant tumor that develops in the skeletal bone tissue is called a "childhood" disease, since the age of 95% of the patients suffering from it does not exceed twenty-five years. The disease is named after the American oncologist, who first described his symptoms in 1921.

Sarkom Ewing, which accounts for up to fifteen percent of all malignant bone tumors in children, is only ahead of the osteogenic sarcoma.

Statistics show that the tumors of this group most often suffer from children and adolescents of the white race. Africans and Asians are less affected by them.

Among fourteen-year-olds, boys are more likely to fall ill (in ratio 1).

Ewing's sarcoma is a diagnosis of an extremely aggressive oncological form of the disease, primarily Affected lower parts of long (tubular) bones, pelvic bones, bone tissues of the spine, ribs, clavicles and blades.

Having penetrated into the bone, the tumor process extremely quickly captures the surrounding soft tissues.

Most often they are localized in the tissues of bones, lungs and bone marrow.

The most sad is the fact that at the time of primary treatment to a doctor at a significant (up to fifty percent) of the number of patients, metastasis is detected, and the presence of micrometastases is observed in even more of cases.

The most characteristic zone of lesion in young (under 20 years old) patients are the tubular bones of the upper and lower extremities. In more mature patients, the bone tissues of the vertebrae, ribs, scapula, skull and pelvis are involved in the pathological process.

Kinds

According to modern classification, there are two types of Ewing sarcoma:

1. A typical Ewing sarcoma, affecting only the bones. This type of sarcoma is most often affected by the iliac bone - the largest element of the human pelvis. Sarcoma (Ewing) of the ilium is observed in almost half the cases of malignant childhood tumors.
2. Ewing's sarcoma of sarcoma, localized in soft tissues.

Causes

The exact cause of Ewing's sarcoma has not yet been established. There is an opinion that the following factors can provoke tumor development:

• Heredity. Ewing's sarcoma is often observed in patients whose family history has had cases of similar illnesses.
• Intrauterine malformation of urogenital organs.
• Young (from five to twenty years) age.
• Belonging to the white race.
• The presence of benign neoplasms in the bones.
• Injuries received in the past.

Symptoms

• Ewing's sarcoma declares itself as a symptom of pain in the place of development of the pathological process. At the initial stage, the pains are of a moderate nature, periodically cease and intensify again. As the tumor develops, the pain syndrome progresses, intensifying toward the night, not stopping either with a bandage or in a state of rest.
• Palpation of the affected area is extremely painful. The area of ​​the skin above the tumor is always hot, reddened and swollen, the subcutaneous veins are enlarged.
• Night pains cause sleep disorders, reduced working capacity, constant fatigue, increased irritability.
• A tumor that develops in the vicinity of the joint, eventually leads to the development of pain syndrome in it, the appearance of low mobility (up to the formation of contracture).
• The pathological process is rapidly progressing. A few months after the first clinical manifestations, the tumor can be not only probed, but also seen with the naked eye.
• At a late stage, a pathological fracture may occur at the location of Ewing's sarcoma.
• In parallel with the development of local clinical manifestations, the symptoms of general oncological intoxication are increasing. Patients feel extremely weak and completely lose their appetite. They have a sharp decrease in body weight (often until complete exhaustion). The raised temperature stays firm at the level of not only subfebrile, but even febrile values.
• At this stage, patients are diagnosed with regional lymphadenitis (inflammation of the lymph nodes). Blood tests reveal anemia.
• A number of specific symptoms depend on the location of the tumor process. If Ewing's sarcoma affected the bones of the lower limbs, lameness develops. Tumor rib can lead to respiratory failure, a pleural effusion and hemoptysis.

Stages of

Ewing tumor in its development is 4 stages:

1. A small new growth appears on the surface of the bone.
2. The pathological process penetrates deep into the bone tissue.
3. Metastasis begins in the nearby organs and soft tissues.
4. Metastases affect distant organs and parts of the body.

Localization

In youngest (younger than twenty years) patients, the disease affects long tubular bones:

• femoral;
• brachial;
• tibial;
• fibular.

In older patients, the pathological process is observed in the flat bones of the ribs, skull, pelvis, scapula, vertebrae.

Ewing's erectile sarcoma affects only soft tissues.

Metastasis

Ewing's sarcoma most often metastasizes into the lungs, less often - into bone tissue and bone marrow.

In later stages, metastases affect the central nervous system.

Cases of metastasis in the pleura, lymph nodes of the mediastinum, other internal organs and into the retroperitoneal space are quite rare.

Due to the very early appearance and rapid progression, the presence of metastases is noted in half of patients who first applied for medical help.

Ways of metastasis:

• hematogenous(through the bloodstream);
• lymphogenous(on the lymphatic vessels);
• retroperitoneal(by retroperitoneal space);
• mediastinal(mediastinum).

Diagnostics

At the initial stage of development of Ewing's sarcoma, doctors of provincial polyclinics, neglecting primary X-ray diagnostics,often take her symptoms for manifestations of a simple inflammatory process, stretching or bruising.

As a result, the treatment strategy turns out to be erroneous, and some therapeutic methods can even contribute to the progression of the disease.

To exclude the possibility of such fatal errors, the detection of malignant neoplasms is carried out by the methods:

• X-ray study. This method is the leading way to detect pathological foci in the tissues of bones.
• Magnetic resonance and computed tomography. This technique is used to determine the stage of the disease, the exact location of its location and the degree of tissue damage.
• Histological examination of affected tissues. Soft and bony tissues are examined by biopsy. The bone marrow samples are examined using bone marrow puncture, trepanobiopsy, and bilateral trepanobiopsy.
• Laboratory blood tests. With Ewing sarcoma, there is a presence of leukocytosis, an increase in the rate of erythrocyte sedimentation, and a high level of lactate dehydrogenase.
• Osteoscintigraphy - studies of bones using radiopharmaceuticals.
• Ultrasonic diagnosis. Such a study will help to identify the presence or absence of metastases in internal organs.
• Molecular genetic diagnosis.

Treatment

The leading methods of treatment of Ewing's sarcoma are:

1. Chemotherapy. Cytostatic preparations (cyclophosphamide, vincristine, adriamycin), which disrupt the growth and fission processes all types of cells introduced into the body of an oncological patient, cope with the smallest metastases. This is facilitated by the specificity of their effect on virtually all tissues and organs of the human body. The destruction of micrometastases slows the growth of the tumor. The introduction of cytostatics is carried out by courses. The duration of breaks between them is from two to three weeks. If metastases were found in the bone marrow, they are exposed to large doses of the above drugs, after which they transplant stem cells.
2. Radiotherapy. High-energy radiation, which is affected by tumor tissue, is repeatedly performed both before and after surgery. Methods of radiation therapy are considered to be the most effective and least traumatic for the patient's body. With their help, you can not only cope with the formation of metastases, but also prevent further relapses of the disease. Dosage of radiation is strictly individual. The duration of one course of radiotherapy is from four to six weeks.
3. Operationalremoval of focusoncological neoplasm. In the course of surgical intervention, the tissues of the ribs, pelvic and femur bones can be partially removed. The area of ​​resection (removal) is determined solely by the degree of prevalence of the tumor process. The removed sites of bones are most often replaced by endoprostheses. Modern surgical interventions rarely end with amputation of the extremities. Surgeons do everything possible to ensure that the patient does not remain disabled.

The prognosis for Ewing sarcoma largely depends on the timeliness of the patient's treatment for a qualified medical that is, from the stage at which the disease was detected and from the degree of prevalence of the pathological process.

For patients who have unresectable (not subject to removal) metastases in the lungs and lymph nodes, the prognosis is extremely unfavorable.

Survival of patients in whom the disease metastasized in bone and bone marrow is not more than thirty percent (under the condition of powerful radiotherapy followed by bone marrow transplantation).

With the timely detection of a tumor (before it spread throughout the body)the possibility of a full recovery is high and is almost seventy percent.

This is facilitated by great success in pharmacology and progress in the methods of treatment. Modern chemotherapy drugs have far fewer undesirable side effects, due to than as a result of complex treatment, doctors manage to prevent the development of many dangerous complications.

It is important not to waste time in the treatment of cancer. If there are even insignificant signs of the development of the tumor process - it is necessary to immediately engage in diagnosis. The patient should know that Ewing's sarcoma is not a verdict.

With the correct treatment tactics and strict observance of the recommendations of a good specialist, a disease can be defeated.

A source: http://gidmed.com/onkologiya/lokalizatsiya-opuholej/kosti-i-myagkie-tkani/sarkoma-yuinga.html

Ewing's sarcoma in children, symptoms, treatment

Ewing's sarcoma in children diagnosed at early stages with complex treatment gives positive results. All patients should be lifelong under the supervision of doctors, to identify new lesions, as well as to monitor the occurrence and development of side effects.

Symptoms

All these symptoms can be harbingers of other diseases, so it is difficult to diagnose the early development of sarcoma.

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Nonspecific manifestations:

1. general weakness;
2. fast fatiguability;
3. sharp weight loss;
4. temperature within 38 degrees;
5. anemia.

Local tissue changes after the affected area (extraosseous sarcoma):

• emerging painful sensations;
• swelling, swelling;
• change of bone relief;
• to the touch the tumor is warm;
• redness of the skin around the hearth;
• inflammation;
• expansion of veins.

If the affected area is the periosteum, the pain manifests itself:

1. weak, but progresses with the development of the disease;
2. disappears, or weakens, but reappears;
3. does not disappear at all (even after rest);
4. does not cease if you fix the joint;
5. increases at night, disturbed sleep;
6. progresses, the movements of the affected joint are limited.

And other characteristic symptoms:

• enlarged lymph nodes, which are nearby.
• fractures arising from the increase in the tumor.
• with defeat of the sarcoma of the spine there is a weakening of the limbs, paralysis.
• if the lower extremities are affected, then lameness appears.
• sarcoma of the bones of the chest manifesting the emergence of problems with breathing, inflammation of the respiratory tract, hemoptysis.
• the occurrence of metastases in nearby tissues, or distant metastases of such organs (liver, lungs, lymph nodes, in the brain).

Diagnostics

Usually the first treatment occurs to the doctor traumatologist, but after the X-ray the patient is referred to an oncologist for all necessary tests and survey. Very often, sarcoma is diagnosed very late, when metastases have already appeared. It is very difficult to detect micro metastases.

To diagnose Ewing's sarcoma, the following studies are carried out:

1. peripheral blood test (for the presence of tumor cells, with a positive result is brain sarcoma);
2. blood test for the presence of: increased ESR, anemia, leukocytosis;
3. biochemical analysis of blood at the level of LDH;
4. Radiography (affected parts of bones, tissues, organs with metastases) detection of sarcoma;
5. X-rays of light;
6. tomography (MRI and CT) of all areas (bones, soft tissues, organs) affected by the tumor;
7. scintigraphy of the skeleton, is carried out in order to reveal the metastases that have arisen; this method allows to reveal the pathology of bones with high accuracy;
8. X-ray examination of the state of the vessels;
9. Ultrasound;
10. a complete examination of the brain (trephine-biopsy);
11. a biopsy of the sarcoma for the final confirmation of the diagnosis;
12. research of tumor tissues (immunohistochemical studies), are a direct confirmation of the diagnosis of sarcoma;
13. DNA samples are taken for research.

There are two stages (stages) of the disease:

• localized form;
• metastatic form.

Localized phasecan be characterized by the presence of a tumor (superficial or left inside the bone tissue) in one place, possibly spreading to surrounding soft tissues.

Metastatic phaseis detected only after the detection of metastases (nearby and distant) on other organs and tissues.

Treatment

For the treatment of Ewing's sarcoma, the following methods:

1. conservative (chemotherapy);
2. conservative (radiotherapy);
3. surgical method (amputation or extensive, partial resection of the affected area of ​​bone and tissue);
4. complex treatment includes all of the above methods.

Chemotherapy- is the introduction of drugs often intravenously, rarely inside. This method of treatment is shown to all patients, regardless of the stage of development of the disease. The course of chemotherapy is 3-4 weeks, several courses are conducted depending on the stage of the disease.

Radiation therapyis carried out externally, to the affected areas. Tumors are sensitive to radiation, at the initial stage of the disease, a complete cure is possible. Radiation irradiation is used in cases where removal of the tumor is impossible.

Surgical methodincludes a complete or partial removal of the tumor, if the operation was carried out on the limbs, ribs, etc. the removed bone is replaced by an endoprosthesis.

Radical removal of the tumor (bone and soft tissue component) is possible if the foci of sarcoma are located on the bones:

• forearm;
• edges;
• peroneal;
• shoulder blades;
• clavicles.

This method reduces the risk of a tumor in the same place. Radiation irradiation is performed on the affected bones, tissues or organs (in particular, the lungs) with metastases.

Forecast

Timely diagnosed disease, as well as the combined therapy performed, gives a good survival result. Patients who have undergone successful treatment can lead a full-fledged lifestyle.

Prognostic factors:

1. the size of the primary tumor;
2. localization of the sarcoma;
3. spread of the tumor;
4. presence of a soft-tissue component;
5. metastasizing process;
6. effective and correctly selected treatment.

After the therapy, a mandatory (lifelong) regular examination is carried out. This is done to identify possible relapses, as well as to monitor the course of side effects.

Side effects can occur immediately during the treatment, as well as after a long period of time.

It can be:

• dysplasia of the bones;
• bone growth can stop, leads to shortening of limbs;
• infertility (male and female);
• the emergence of secondary tumors;
• the appearance of malignant tumors;
• on the overall development of the child.

Patients who have an unfavorable prognosis (metastases) undergo chemotherapy with mega doses and total body irradiation. Patients with bone marrow or bone metastases perform bone marrow and stem cell transplantation, this method can cure more than 30% of patients.

An unfavorable prognosis is for patients diagnosed with sarcoma:

1. pelvic bones;
2. the tissue of the retroperitoneal space;
3. abdominal cavity;
4. cavity of the small pelvis.

An unfavorable prognosis is associated with the presence in the above areas of the body: tumors of large sizes, and also the least possibility of resectability, low sensitivity to chemo and radiation therapy.

The course of detection and treatment of Ewing's sarcoma in children is directly influenced by its location, size, and the chosen therapy.

The average age of patients (Caucasoid race) is 10 years, the sexual discrimination of patients begins after 14 years (boys fall 2 times more often).

To date, the etiological components of the origin of Ewing's sarcoma have not been established.

A source: http://rak.hvatit-bolet.ru/vid/sarkoma/sarkoma-juinga-u-detej.html

Ewing's sarcoma

Ewing's sarcoma- malignant neoplasm developing from bone tissue. Most often occurs in adolescence or adolescence. It affects flat and long tubular bones.

It is one of the most aggressive tumors, prone to early metastasis. The cause of development is not exactly established, however, there is a definite connection with previous injuries and some skeletal anomalies.

It manifests itself with pain, then with swelling, local hyperemia and hyperthermia, as well as local expansion of the venous network. In later stages, the tumor is determined, often in combination with a pathological fracture of the affected bone.

Combination treatment includes surgery (if possible - radical removal of the neoplasm) in combination with radiation therapy, as well as pre- and postoperative chemotherapy.

It is accompanied by pain and local changes in soft tissues (edema, hyperemia, hyperthermia). In later stages, the tumor becomes noticeable upon examination and palpation.

Treatment includes prompt removal of neoplasm, chemotherapy and radiation therapy.

Ewing's sarcoma is the second most frequent malignant tumor of bone tissue that occurs in childhood, it ranges from 10 to 15% of the total number of malignant bone tumors.

In adults over 30 and children under 5 years of age is rare. The greatest number of cases falls on the age of 10 to 15 years.

Boys are sick more often than girls, Caucasians are more often representatives of Negroid and Mongoloid races.

In some cases, the primary neoplasm in this disease occurs in soft tissues, and not in bones. This form of the disease is called Ewing's extra-osseous (extra-osteal) sarcoma.

In addition, there are similar in nature and structure aggressive malignant tumors, belonging to the group of so-called tumors of the Ewing's sarcoma family. In this group, in addition to the extraosseous and bone sarcoma, they include PNEO (peripheral primitive neuroectodermal tumors).

The extra-osophageal forms and the ESRO total about 15% of the total number of cases of Ewing's sarcoma.

Causes and predisposing factors of Ewing sarcoma development

The reasons for the development of Ewing's sarcoma are not exactly established at the present time. However, a number of researchers believe that a certain role in the onset of this disease is played by a hereditary predisposition. Approximately in 40% of cases, a connection with anterior trauma is revealed.

There is evidence of an increased likelihood of developing the disease in patients with some types of skeletal anomalies (aneurysmal bone cyst, enchondroma, etc.), as well as violations of the intrauterine development of the genitourinary system (reduplication of the renal system, hypospadias).

There is no connection with the effect of ionizing radiation.

The first, the earliest sign of the disease is pain in the area of ​​injury. Initially, it is weak or moderate, spontaneously weakens or disappears, and then appears again.

On the contrary, Ewing's sarcoma is characterized by increased pain at night.

As the progression of pain becomes more intense, deprive sleep and limit daily activity.

In the nearby joint develops painful contracture. Palpation of the affected area is painful. The temperature of the skin above it is increased.

There is a pastovozhnost soft tissue, local hyperemia and the expansion of the subcutaneous veins.

The tumor rapidly increases and (usually several months after the onset of the first symptoms) becomes so large that it can be palpated. In later stages of the neoplasm, a pathological fracture often occurs.

Local clinical signs are combined with growing symptoms of general tumor intoxication. Patients complain of weakness and loss of appetite.

There is a decrease in weight until exhaustion. Body temperature is increased to subfebrile or even febrile digits. Regional lymphadenitis is determined.

The blood test reveals anemia.

Some symptoms depend on the localization of the neoplasm. So, in the occurrence of Ewing's sarcoma, lameness appears on the bones of the lower limbs.

With neoplasm in the region of the bones of the chest, respiratory failure, pleural effusion, and hemoptysis may occur.

Localization and metastasis of Ewing's sarcoma

Most often Ewing's sarcoma is revealed in the femur, pelvic bones, tibial and fibular bone, scapula, ribs, humerus and vertebrae.

When originating in long tubular bones, the tumor usually localizes in the diaphysis region, and then spreads toward the epiphyses as it grows.

In more than 90% of cases, the intramedullary location of the neoplasm and the tendency to spread the tumor cells along the medullary canal are noted.

Ewing's sarcoma most often metastasizes into the lungs. In second place in the prevalence - metastases in the bone marrow and bone tissue.

In the late stages, almost all patients are diagnosed with metastases to the central nervous system.

In rare cases, distant metastases are detected in the pleura, lymph nodes of the mediastinum, internal organs and retroperitoneal space.

Because of early metastasis, at the time of diagnosis, 15 to 50% of patients already have metastases that can be detected using routine research. The overwhelming number of patients have micrometastases.

As a rule, at the initial stage, patients turn to traumatologists. And the first study, which allows to suspect Ewing's sarcoma, is the radiography of the affected bone.

For this pathological process, a combination of reactive and destructive processes of bone formation is characteristic.

The contours of the cortical layer are indistinct, the stratification and disintegration of the cortical plate is determined.

In addition, the pictures show the area of ​​soft tissue changes, in size larger than the primary bone tumor.

In this case, the soft-tissue tumor component is characterized by homogeneity, cartilaginous inclusion, foci of calcification or pathological bone formation are absent.

When identifying typical X-ray signs of Ewing's sarcoma, the patient is referred to a department oncology, where an extensive examination is conducted to assess the condition of the primary focus and identify metastases.

During such a survey, a CT scan or magnetic resonance imaging of bones and soft tissues affected by a malignant process is performed.

These studies can accurately determine the size of the neoplasm, the extent of its spread along the bone marrow channel, the connection with the neurovascular bundle and surrounding tissues.

For the detection of metastases in lung tissue, computed tomography and lung radiography are used. To detect metastases in the bones, bone marrow and internal organs - positron emission tomography, ultrasound and osteoscintigraphy.

In addition, a number of studies are performed to accurately assess the nature of the lesion.

Since Ewing's sarcoma is characterized by local and distant damage to the bone marrow, The bilateral trepanobiopsy, during which the bone marrow is collected from the iliac wings bones.

To assess the nature of the process, immunohistochemical and molecular-genetic research (fluorescent hybridization - to confirm the diagnosis, polymerase chain reaction - to identify micrometastases).

Treatment of Ewing's sarcoma

Since Ewing's sarcoma belongs to the category of highly aggressive tumors, early giving metastases, its treatment should include the impact on the whole body, and not only on the primary focus, even in cases where metastases were not detected.

The fact is that there is a very high probability of early appearance of micrometastases, which at the time of diagnosis can not be detected using existing methods.

Treatment of Ewing's sarcoma is combined, includes both conservative therapy and surgical interventions and consists of the following components:

• Pre- and postoperative chemotherapy, usually with the use of several drugs (doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide and actinomycin in various combinations). After the course of treatment, the tumor response to therapy is evaluated. A good result is the presence of not more than 5% of living tumor cells.
• Radiation therapy with high doses. It is carried out on the primary focus, and, in the presence of metastases in the lung tissue - and on the lungs.
• Surgery. If possible, the tumor is removed radically, along with the soft-tissue component. In this case, the removed bone site is replaced by an endoprosthesis. But even if, due to the location and size of the lesion, it is not entirely possible to remove it completely, operative treatment (partial resection) allows to improve control over the tumor and increase the chances for a successful Exodus.

In the past, Ewing's sarcoma usually carried out crippling operations - amputations and exarticulations.

Modern techniques allow performing organ-preserving surgical interventions not only on small (peroneal, radial, ulnar, scapula, collarbone and ribs), but also on large bones (humerus, femoral and even bones of the pelvis).

When metastasizing to the bone marrow and bones, intensive therapy is prescribed, which includes total irradiation of the whole body, chemotherapy using megadoses of drugs and transplantation of peripheral stem cells or bone the brain.

Prognosis for Ewing sarcoma

Timely combination treatment provides a 70% overall survival of patients with localized Ewing's sarcoma.

In the presence of metastases in the bone and bone marrow, the prognosis is significantly worsened, but the combination of high-dose chemotherapy, total radiation therapy and bone marrow transplantation provides an opportunity to increase the survival rate of patients with metastatic disease from 10% to 30% and more.

All patients after recovery should undergo a regular examination for early detection of relapses and control over side effects.

It should be borne in mind that a number of side effects can occur not only during the course of treatment, but also after a long time after its termination.

Such effects include male and female infertility, cardiomyopathy, dysplasia of bones and an increase in the likelihood of developing secondary malignant neoplasms. However, many patients who have been treated for Ewing's sarcoma can live a full life.

A source: http://www.krasotaimedicina.ru/diseases/traumatology/ewing-sarcoma

Ewing sarcoma: diagnosis and stages of the disease

One of the most aggressive malignant diseases is Ewing's sarcoma. This type of tumor was discovered at the beginning of the 20th century.

But until now the disease is considered poorly studied.

Well-known oncologist James Jung singled out this kind of sarcoma as a separate group because he has some peculiarities.

The tumor is most often affected by tubular bones, pelvis, spine.

Until recently, 90% of patients developed metastases rapidly, spreading 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 an unfavorable prognosis.

A feature of Ewing's sarcoma is also that the location of the tumor localization depends on the age of the patient.

Very rarely, but only soft tissues, such as the lungs, are affected.

Cause of disease

Despite many years of research into the disease, the cause of tumor development has not yet been determined. But scientists have identified several predisposing factors to the appearance of the disease:

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

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

How is the disease manifested?

The main symptoms of Ewing's sarcoma are pain in the bones. At first they can be weak, aching, intermittent for a while. Then they become more intense.

The peculiarity of them is that they do not subside, but only intensify at night or when the affected limb is immobilized.

This they differ from pain in trauma and inflammatory diseases.

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

Over time, the tumor seizes surrounding soft tissue. Develops a strong edema with hyperemia of soft tissues, which become densified, hot to the touch.

There are also symptoms of intoxication: fever, nausea, weakness, weight loss, lymphadenopathy, lack of appetite, anemia.

In the course of the development of the disease, its external signs are increasingly manifesting: the tumor grows, the skin over it becomes crimson or cyanotic, thinens, veins start to stand out. Because of the 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 can be hemoptysis, shortness of breath.

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, according to which treatment is prescribed

Stages of development of the disease

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

• Stage 1 is a small tumor on the surface of the bone, outwardly invisible, the disease proceeds almost asymptomatically;
• 2 stage - the neoplasm develops deep into the bone tissue, affects the surrounding muscles and tendons, severe pains appear, external changes in soft tissues are visible;
• Stage 3 - there are metastases in the nearest organs and tissues, the tumor itself is already very noticeable, and the pains prevent 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 the correct diagnosis, it is necessary for the child to see a doctor for examination if symptoms of anxiety appear. Diagnosis of the disease includes such methods:

1. first of all, 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;
2. to confirm the diagnosis is shown MRT of bones and soft tissues, which will show the boundaries of the neoplasm, the extent of its spread, the defeat of nerves, vessels;
3. also a computed tomography of the lungs is done to determine the presence of metastases;
4. sometimes they are prescribed osteoscintigraphy, ultrasound, angiography, positron emission tomography;
5. To more accurately determine the type of tumor, biopsy, bone marrow examination, immunological and genetic analyzes are done.

Such 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 for more effective treatment.

Various diagnostic methods help in time to correctly diagnose

How to treat Ewing's sarcoma

This disease is very difficult to diagnose at the initial stages. Usually, when diagnosed, the tumor has already metastasized.

But with timely treatment, you can completely eliminate the pathological focus, prevent metastases, and exclude the possibility of relapses. But most often the prognosis for patients is unfavorable. Complete recovery 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 the metastases spread to the bone marrow.

The most radical and effective method of treating 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 recovery and survival of patients depend, first of all, on its timeliness. The earlier a patient goes to a medical facility, the faster his condition can be alleviated.

Tumor therapy must necessarily be carried out in a specialized clinic, where a complex treatment is prescribed, including several methods.

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

Surgical removal of tumor

This is the most effective method of treating the disease.

Depending on how the tumor spreads and its metastases, not only the affected areas can be cut out during the operation.

Pelvic or femoral bones, ribs, other parts of the skeleton, surrounding soft tissues are often removed. Endoprostheses are placed on the site of the removed bones.

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

Radiation therapy

Used to treat high doses of radiation - 4000-5000 R. The effect is on the primary focus of the neoplasm, and if the metastases spread into the lung tissue, then the lungs.

Side effects of such treatment can be headaches, restriction in the movement of joints, in children - it slows the growth of bones and mental disorders.

But, despite this, radiotherapy is now the most effective and safe method of treating the disease.

Chemotherapy for Ewing sarcoma

Such treatment is effective because drugs can destroy even the smallest metastases that can not be diagnosed. The most common drugs for chemotherapy are "Cyclophosphan "Adriamycin "Vincristine "Ifosfamide "Vepesid".

They are prescribed most often intravenously, monthly courses with small - 2-3 weeks - interruptions. Such treatment usually lasts 10-12 months. Often different drugs combine with each other.

Chemotherapy is used in the initial stages, as well as before and after surgery.

And still we advise you to read:Common diseases of leg joints

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

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

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

The success of recovery depends not only on proper treatment, but also on the patient's own psychological state

What does the recovery depend on?

The success of treatment depends, first of all, 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.

It is necessary only in time to apply to a medical institution, to comply with all the doctor's recommendations and to believe in recovery. The psychological mood is very important, as well as the support of close people.

Now more than 70% of patients after recovery get a chance for a normal life without relapse.

In 90% of cases, metastases spread, most often to the bone marrow and lung tissue. And in the late stages, almost always the tumor metastasizes into the central nervous system.

Therefore, it is very important to start treating the disease as early as possible.

Even after recovery, patients need a follow-up to see the progression of the disease recurrence and prevent complications. Most often it is for life. Only a small percentage of patients after recovery can return to full-fledged life.

Ewing's sarcoma is a very dangerous disease that affects children and adolescents.

The further life and health of such patients depends on how parents noticed the first symptoms of the disease in time, how the doctor's recommendations followed, 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 medical development makes it possible for 70% of patients to fully recover.

A source: http://MoyaSpina.ru/bolezni/sarkoma-yuinga