Ewing’s sarcoma: what disease is this and what is its prognosis?
Modern diagnosis and treatment of Ewing’s sarcoma
Bone tumors have a special place in Oncology, as accompanied by great difficulties in the process of diagnosis and treatment.
Ewing’s sarcoma is an extremely malignant tumor.
Its frequency is about 9% of all malignant bone tumors, which is 3 times more common than osteosarcoma.
Mainly observed in children and adolescents. Women are sick 2 times less often than men.
As an independent nosological form sarcoma was highlighted by Ewing in 1921. Emphasizing the origin of the tumor from the vascular endothelium, the author called her endothelioma. Currently, the debate about the nature of education continue.
This type of sarcoma develops from reticuloendothelial tissue and consists of cells that are in the process of its development is not differentiated and have not acquired the characteristic properties.
Depending on which tissue (bone, cartilage, connective) predominates in the tumor, is isolated:
- fibroplastic sarcoma.
The first of them bears the name of Ewing’s tumor.
The characteristic localization of osteogenic sarcoma are the diaphysis of long bones. The most commonly affects the femur and the tibia. Much less likely to suffer the wings of the iliac bones, scapula, vertebral bodies.
Mostly in the pathological process involves only one bone. The lesions drop-out rates of a tumor can be detected in liver, lung, lymph nodes.
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The reasons and mechanisms of occurrence of the bone tumors to date not fully understood. Most scientists adhere to the theory of multistage-stage carcinogenesis.
According to this opinion the tumor arises in the process of sequential accumulation of various damage of the genetic apparatus of the cell.
These changes can occur under the influence of physical (radioactive and ultravioletovo radiation), chemical (nitrosoedinenii, polycyclic carbohydrates, cytostatics, heavy metals, benzene and its derivatives), Biologicheskie (human papilloma virus, Epstein-Barr, hepatitis b, HIV)
The clinical picture
Ewing’s sarcoma can often have symptoms that vary greatly in different cases.
Often, the disease may simulate chronic osteomyelitis. In most cases, onset of the disease erased. The main complaint that leads the patient to the doctor is pain.
Pain syndrome is fairly intense (but less intense than under osteogenic sarcoma), even alone, in the absence of loads.
The pain is wave-like in nature. The tumor is characterized by rapid growth. When you increase the size of the pathological focus increased pain, there is swelling of the surrounding tissues.
As a result of venous congestion of the superficial veins expand and the skin becomes thinner, acquires a bluish color.
After 4-5 months of onset of the movement at the nearest joint is gradually reduced, until the development of painful contractures.
The limb loses its support function. This symptom is typical for osteosarcoma. In the case of Ewing’s tumor presents a clinical picture evolves much more slowly.
Palpation of the tumor to determine its dense texture, in some cases, can determine the foci of softening. This symptom is a result of the collapse of the affected tissues. In the later stages of the disease, palpation of education may appear crunch.
In most cases, swollen lymph nodes are not observed. In the initial stages the General health of the patient does not change. Only after 3-4 months when the tumor reaches considerable size, the body temperature rises to 39 0C.
Some authors distinguish two variants of the disease.
- In one case the tumor for a long time remained unnoticed, metastases appear later.
- In another case, the disease progresses rapidly, causes destruction of bone, the tumor metastasizes early.
Features of clinical protocols of examination and treatment
Before the appointment of treatment the patient must undergo comprehensive examination:
- General clinical analysis of blood, urine;
- biochemical analysis of blood;
- determination of the blood group, Rh factor;
- a blood test for syphilis;
- roentgenography of organs of a thorax;
- x-ray of affected limb segment;
- Ultrasound examination of abdominal cavity;
- biopsy of the tumor with subsequent morphological study (subject to availability);
- computed tomography of the pathological education;
- consultation of narrow specialists (to correct for any special treatment).
Only after a comprehensive examination at the conclusion of the consultation the patient is sent for a specific or symptomatic treatment.
Laboratory blood tests may reveal increased number of white blood cells, ESR acceleration. In the later stages of the disease anemia. When biochemical analysis of blood and urine there is an increase in alkaline phosphatase, which is characteristic of a malignant process.
In the beginning of its development on the radiograph the tumor is blurred and fuzzy outlines. Characteristic osteoporosis in the form of scattered spots, the destruction of a cortical layer of tubular bones.
With the defeat of the periosteum (accompanied by a sharp increase in pain) there is a characteristic radiographic sign is the «visor».
In the process of disease progression, a «peak» increases, there is a bulbous figure, narrows the medullar canal.
X-ray of the variety of Ewing’s sarcoma:
- small focal diffuse destruction of bone tissue oval or round shape;
- longitudinal dissociation and separation of the cortical layer with the formation of lamellar destruction;
- large pockets of destruction of bone.
Microscopically the tumor tissue represented by monotonous small cells with round nucleus and ill-defined cytoplasm.
The nucleus of the cell is brightly painted, occupies almost the entire cell. The extracellular matrix is not defined. Cell division a few.
Differential diagnosis of
To differentiate Ewing’s sarcoma is necessary chronic osteomyelitis, periostitis, osteosarcoma, bone metastases and lymphoma.
The stage of the disease
Ewing’s sarcoma develops in 4 stages:
- Stage I. Patients complain of pain. Visually, the limb is not modified. On the radiograph the tumor appears like a fuzzy focus of osteoporosis sclerotic inclusions, the surrounding bone tissue is not changed.
- Stage II. The pain intensifies. The limb in the region of the tumor becomes edematous, cyanotic. On the radiograph there appears a symptom of the «hood».
- III — IV stage. The pain becomes unbearable. Pathological education can be palpated, it can be determined visually. The skin over the tumor begins to Shine. Limb function is severely disrupted.
Possibilities of modern medicine
The treatment of Ewing’s sarcoma is the combination of chemotherapy and radiotherapy, as determined by high sensitivity of the tumor to these factors.
The greatest efficiency has exposure to a total dose 4000-5000 R. Surgical treatment because of their biological characteristics relegated to the background.
The operation is usually performed in a volume of the amputation of the limb.
This treatment is possible only under conditions of compliance with the rules and ablastic antiblastic.
However, surgical treatment can not prevent the occurrence of metastases.
At the present stage of development of medicine is developing new chemotherapeutic agents, allowing to avoid surgical intervention.
The course of the pathological process is fairly quick. Metastases occur in the first year of the disease. Even under conditions of timely treatment of the disease the prognosis for life should give careful.