Ewing's Sarcoma In Children - Causes, Symptoms And Treatment

2024 Author: Josephine Shorter | [email protected]. Last modified: 2023-12-16 21:43

Ewing's sarcoma

Ewing's sarcoma is a malignant neoplasm that affects the human skeleton. Usually, the tumor begins to develop on the lower parts of the long bones, pelvic bones, ribs, spine and collarbone. The tumor spreads rather quickly to the adjacent soft tissues.

Ewing's sarcoma is one of the most aggressive types of malignant tumor. Approximately 90% of patients developed metastases prior to therapy. Most often, metastases occur in the bone marrow and organs such as the lungs, liver, as well as the lymphatic system and bones. Many patients have micrometastases. It happens that Ewing's sarcoma is extraosseous and affects soft tissues.

This oncological disease is the second most frequent disease, occurring mainly in childhood. In people over 30 and in children under the age of 5, this disease is quite rare. Most often appears in children from 10 to 16 years old. At the same time, it was noticed that boys suffer from this disease much more often than girls, and representatives of the Caucasian race are more susceptible than people with dark skin.

Content:

• Causes of Ewing's sarcoma
• Symptoms of Ewing's sarcoma
• Diagnosis of the disease
• Disease stages
• Localization and metastases of the disease
• Ewing's sarcoma treatment
• Predisposing factors for the onset of Ewing's sarcoma
• Prevention of Ewing's sarcoma

Causes of Ewing's sarcoma

Until now, the exact causes of the appearance of this disease have not been established, but researchers have put forward several theories that have certain justifications. Some scientists believe that an inherited predisposition plays an important role in the development of Ewing's sarcoma in humans. There were cases when the disease was found in children born in the same family.

There is also an opinion that Ewing's sarcoma is a consequence of the trauma suffered. The difficulty in confirming this theory lies in the fact that the time interval from the moment of injury to the onset of tumor development is quite large. Perhaps the trauma only draws attention to the part of the body where there was already a malignant neoplasm.

There is evidence that there is a higher likelihood of developing Ewing's sarcoma in people with skeletal abnormalities, such as bone cyst, enchondroma, and others, as well as intrauterine development of the genitourinary system.

The essence of another theory is that the cause of the development of this disease is chromosomal changes that cause dysfunctions of certain genes. The relationship of this disease with the impact on the body of harmful environmental factors and radiation has not been established.

Symptoms of Ewing's sarcoma

The first sign of this disease is pain in the area of tumor localization. At first, the pain is mild and may suddenly disappear. Unlike pain accompanying inflammatory processes or injuries, discomfort does not subside with fixation of the limbs, and also intensifies at night. As the tumor grows, the pain increases, interfering with sleep and greatly limiting the person's activity. Palpation (palpation) of the affected area is especially painful. The area of the skin located above the tumor has a higher temperature, redness appears, the saphenous veins noticeably expand, and the tumor becomes visible.

The tumor grows very quickly and several months after the first signs are found, it can be felt and identified visually. Symptoms of general intoxication of the body also appear: weakness, lack of appetite, weight loss, up to exhaustion. Body temperature is kept at a high level. A blood test often reveals anemia.

Together with the increase in the tumor, the functions of the nearby joints suffer, their motor ability decreases. In the later stages of development, the tumor can cause a pathological bone fracture. When the tumor is localized on the tubular bones of the lower extremities, gait disturbance and lameness may appear. If the spinal column and its tissues are affected, the functioning of the pelvic organs is disrupted, manifested as incontinence of urine and feces, in the later stages - paralysis of the limbs. If the skeleton of the chest is damaged, breathing disorders, hemoptysis may appear.

Diagnosis of the disease

There are frequent cases when at the initial stage of the disease, patients with similar symptoms turn to traumatologists. Thus, the first study to help make assumptions about the diagnosis is bone x-rays. In this case, you can notice pathology in the process of bone formation. As a rule, the contours of the cortical layer are indistinct; stratification is noticeable on the cortical plate. Bone growths and significant changes in soft tissue are also visible. If signs of oncological disease are detected at this stage, the patient is sent to the oncology department for an extended examination of the tumor focus and the degree of metastasis.

At the next stage of the examination, computed and magnetic resonance imaging are performed. With the help of these studies, it is possible to detect metastases and assess the general condition of the bone marrow canal, as well as determine how badly the soft tissues around the tumor focus are affected and find out the location and size of the neoplasm.

A biopsy is used to clarify the nature of the neoplasm and the stage of the disease. For this, material is taken from a site of bone tissue located in close proximity to the medullary canal, and if this is not feasible, from soft tissue, which is also affected by sarcoma. Immunohistochemical and histological analyzes will help determine chromosomal abnormalities.

Since Ewing's sarcoma is characterized by metastasis to the lungs, tomography and x-rays of the chest area are performed, which can confirm the presence of metastases. Metastatic bone lesions can be detected during osteoscintigraphy.

It is also recommended to conduct a study of the bone marrow material to determine the presence of a distant or local lesion. This is done using bilateral trepanobiopsy. Also, the state of the bone marrow will help determine bone marrow puncture and trepanobiopsy.

Laboratory studies are also carried out to determine changes in the composition of the blood, in which attention is paid to the level of leukocytes and lactate dehydrogenase. The latter indicator is increased in patients with a particularly aggressive course of the disease.

Disease stages

Based on the degree of prevalence, there are 2 main stages of sarcoma: localized and later - metastatic. The first stage has one tumor focus, with the tumor spreading to nearby soft tissues. The second stage is characterized by the presence of several foci, which may be distant from each other.

A more accurate classification consists of 4 stages:

• a relatively small neoplasm on one of the bones;
• a tumor deeply embedded in the bone tissue;
• metastasis to adjacent tissues and organs;
• distant from the primary focus.

The first two stages are localized, the third and fourth are metastatic.

Localization and metastases of the disease

Most often, this cancer begins to develop in the pelvic bones, femurs, tibia, ribs, shoulder blades, humerus and vertebrae. The neoplasm is localized in the area of the diaphysis, after which it spreads to the area of the epiphyses. There is a high tendency for affected cells to enter the medullary canal.

Tumor metastases most often appear in the lungs, slightly less often in bone tissue and bone marrow. In the event that the disease has entered a late stage, the likelihood of metastasis in the central nervous system is quite high.

Since metastases appear quite early and progress quickly, by the time the patient turns for help and the diagnosis is made, about half of the patients already have metastases located in various tissues and organs of the body. They can be detected during radiography. In many, micrometastases are found - neoplasms of microscopic size, which are almost impossible to detect by known methods.

Metastases are spread in the following ways:

• through blood flow;
• through the lymphatic vessels;
• along the mediastinum;
• in the retroperitoneal space;

Most often, metastases spread to other organs with blood. The lymphogenous variant indicates a severe degree of the disease and makes it possible to make unfavorable predictions.

Ewing's sarcoma treatment

Due to the fact that this disease is accompanied by metastases, its treatment should be carried out systemically and include polychemotherapy, which will affect the entire patient's body. If this is not done, there is a high likelihood of relapse. They also provide radiation therapy and surgical treatment. Usually surgery - removal of the tumor - is the most effective way to defeat the disease.

If the limbs are affected, the surgeon removes a portion of the bone, replacing it with an endoprosthesis. In especially difficult cases, limb amputation may be required, although, given the level of modern medicine, this is used less and less. After the tumor is removed, they get rid of the malignant cells located around the site of its localization, using radiation therapy for this purpose. In situations where it is not possible to remove part of the bone, the tumor is treated only with radiation therapy, but then the chance of recurrence is much higher.

In parallel with radiation and surgical intervention, chemotherapy is also used. To determine the method of treatment, specialists examine the particles of the tumor. On average, a course of treatment with chemicals for a disease such as Ewing's sarcoma lasts from 7 to 10 months. When it comes to the form of the disease accompanied by metastases, patients undergo high-dose chemotherapy treatment.

The statistics of the five-year survival rate of patients, given the localized form of sarcoma, shows that about 70% of those who survived the disease survive, but this is possible only in the case of systematic and timely treatment started. If bone marrow metastases develop, even such effective high-dose chemotherapy can only provide a 25% survival rate. The further fate of the patient depends on the degree of tumor sensitivity to chemicals, as well as on the nature of its localization.

After a complete recovery from this cancer, it is strongly recommended to undergo regular examinations, this will also allow you to track the appearance of side effects. Despite how long ago the disease was transferred, such people should be observed by a doctor for life. If arthroplasty has been performed, as a rule, the patient needs long-term rehabilitation. In cases of forced limb amputation, the rehabilitation period takes longer.

Radiation therapy and chemotherapy can have consequences such as impaired bone growth, cardiomyopathy, and loss of fertility. In addition, there is a risk of secondary oncological tumors. However, there are patients who have successfully completed their treatment and returned to their normal lifestyle.

Predisposing factors for the onset of Ewing's sarcoma

• age - from 10 to 16 years, but the disease was diagnosed in persons from 5 to 30 years old;
• male;
• bright skin;
• injuries that caused bone damage;
• any disturbances in the development of the skeleton;
• anomalies in the structure of the genitourinary system;
• benign tumors on bone tissue;
• the presence of cancer in the immediate family;
• poor heredity, in particular genetic disorders.

Prevention of Ewing's sarcoma

Unfortunately, at this stage in the development of medicine, there are no ways to prevent this disease, like many other tumors in children. Scientists were able to establish that the lifestyle of parents and children does not affect the possibility of this disease.

The author of the article: Bykov Evgeny Pavlovich | Oncologist, surgeon

Education: graduated from residency at the Russian Scientific Oncological Center. N. N. Blokhin "and received a diploma in the specialty" Oncologist"