Sarkomu Ewing - a malignant tumor that develops in the skeletal bone tissues, is called a "childhood" disease, since the age of 95% of 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.
Sarkomu 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 the fourteen-year-olds, boys are more likely to fall ill( in a ratio of 1.5: 1).
What is Ewing's sarcoma?
Ewing's sarcoma is a diagnosis of an extremely aggressive oncological form of the disease, primarily affecting the lower parts of long( tubular) bones, pelvic bones, bone tissues of the spine, ribs, collarbones and scapula.
Having penetrated into the bone, the tumor process extremely quickly captures the surrounding soft tissues. The rate of development of the tumor is so high that
soon after the appearance of the first symptoms in patients develop metastases .Most often they are localized in the tissues of bones, lungs and bone marrow.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.
Types of
According to modern classification, two types of Ewing sarcoma are distinguished:
- Typical Ewing sarcoma , affecting exclusively 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.
- Injected sarcoma of Ewing , localized in soft tissues.
Reasons for
The exact cause of Ewing's sarcoma is not yet 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 old) age.
- Belonging to the white race.
- The presence of benign neoplasms in the bones.
- Previous injuries. Symptoms of
- 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 skin area above the tumor is always hot, red and swollen, the subcutaneous veins are enlarged.
- Night pains cause sleep disturbances, reduced performance, constant fatigue, increased irritability.
- A tumor that develops close to the joint, eventually leads to the development of pain syndrome in it, the appearance of inactivity( 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.
- In the late stage of the localization of Ewing sarcoma, a pathological fracture may occur.
- 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 swelling in its development goes through 4 stages:
- A small new growth appears on the surface of the bone.
- The pathological process penetrates deep into the bone tissue.
- Metastasis begins in nearby organs and soft tissues.
- Metastases affect distant organs and areas of the body.
Localization of
In youngest( younger than twenty years) patients, the disease affects long tubular bones:
- femoral;
- shoulder;
- tibial;
- peroneal.
In patients of older age, the pathological process is observed in the flat bones of the ribs, skull, pelvis, scapula, vertebrae.
Ewing's intestinal sarcoma affects only soft tissue.
Metastasis
Ewing sarcoma most often metastasizes into the lungs, slightly 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.
Routes of metastasis:
- hematogenous ( through the bloodstream);
- lymphogenous ( by lymphatic vessels);
- retroperitoneal ( by retroperitoneal space);
- mediastinal ( mediastinum).
Diagnosis
At the initial stage of development of Ewing's sarcoma, doctors of provincial polyclinics, neglecting primary X-ray diagnostics, often take its symptoms for manifestations of the simple inflammatory process of , stretching or bruising.
As a result, the treatment strategy is erroneous, and some therapeutic methods may even contribute to the progression of the disease.
To exclude the possibility of such fatal errors, detection of malignant neoplasms is performed using the methods:
- X-ray study. This method is the leading way to detect pathological foci in the tissues of bones.
An X-ray photo depicting Ewing's sarcoma of the hand of
- 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 - bone research using radiopharmaceuticals.
- Ultrasonic diagnosis. Such a study will help to identify the presence or absence of metastases in internal organs.
- Molecular genetic diagnosis.
Treatment of
The leading methods of treatment for Ewing's sarcoma are:
- Chemotherapy. Cytotoxic drugs( cyclophosphamide, vincristine, adriamycin), which disrupt the growth and division of 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.
- 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.
- Operative removal of the focus of oncological 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.
Survival prognosis
The prognosis for Ewing's sarcoma depends to a large extent on the timeliness of the patient's treatment for qualified medical care, 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 full recovery is high and is almost seventy percent .
In the treatment of cancer, it is important not to miss the time. 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 right treatment tactics and strict observance of the recommendations of a good specialist, an ailment can be defeated.
A video on symptoms and treatments for Ewing's sarcoma: