Ewing's sarcoma is a dangerous cancer disease in which cancer cells develop in bone tissue. Most often (in 70% of cases), this disease affects the bones of the lower extremities. The Ewing sarcoma code for the ICD is 10 C40, C41.
The disease got its name by the name of the doctor James Ewing, who discovered this pathology in 1921. In his works, James described the disease as a tumor that occurs primarily in the tubular long bones.
Ewing's sarcoma: what is this disease?
Of all existing oncological diseases, this pathology is considered one of the most dangerous. This is explained by the high aggressiveness of the tumor: it progresses rapidly and gives multiple metastases.
The main group of patients with this diagnosis are children aged 10-20 years. In adults, Ewing's sarcoma is also diagnosed, but much less frequently. Moreover, in boys this type of tumor is more common than in girls. To date, doctors can not explain this dependence on gender.
The clinical and morphological symptoms of bone cancer are very similar to the manifestation of a squamous cell soft tissue tumor, the only difference is the localization of pathological cells. For this reason, a tumor affecting the skeleton is called Ewing's classic sarcoma, and one that occurs in the soft tissues of the chest, spine and retroperitoneal space is called extra skeletal sarcoma.
Causes of the disease
The exact reasons for the development of sarcoma of this type could not be found. However, leading oncologists put forward a number of assumptions that are based on an analysis of disease statistics.
- Genetic predisposition. Ewing's sarcoma in children is often diagnosed in several sisters and brothers, which became the reason for the theory of predisposition to bone sarcoma.
- Bone injuries. In approximately 40% of cases, the tumor focus is located in the place where the injury was earlier (fracture, severe bruise).
- Some skeletal abnormalities. Presumably, the risk of developing sarcoma is affected by the presence of skeletal abnormalities, including aneurysmal bone cyst and enchondroma.
- Pathologies and abnormalities in the genitourinary system. Among them: reduction of the renal system or hypospadias.
It is important to note that the presence of one or another factor does not mean that a person will develop a cancerous tumor over time. The presence of these signs only increases the risk of developing the disease.
Risk factors
There are several risk factors that require increased attention:
- male - in girls and women, Ewing's sarcoma is detected much less frequently;
- age 10-20 years - the majority of patients (64%) belong to this age group;
- race - owners of white skin suffer from bone sarcoma much more often than blacks.
Pathogenesis (what happens during an illness)
The tumor in this cancer is represented by small round cells that have divisions in the form of fibrous layers. Distinctive features of the cell structure can be called the regular shape, the presence of oval or round nuclei with basophilic nucleoli and chromatin. The tumor does not produce new bone tissue.
What kind of disease is Ewing's sarcoma, and how does it develop? Mostly, neoplasm growth occurs due to uncontrolled cell division. According to scientists, such a pathology is provoked by a special type of genetic damage. Among these lesions is a mutation of a gene responsible for coding for the production of a receptor capable of transforming a growth factor. As a result, enhanced division of pathological cells is not blocked.
Types of disease
In medicine, Ewing's sarcoma is divided into 2 types:
- localized - a neoplasm that affects a certain area of bone tissue and possibly spreading to the nearby lymph nodes and tendons;
- metastatic - sarcoma, which spread throughout the body and affects the bones, lymphatic system, blood vessels, as well as distant internal organs.
Stages of the development of the disease
In addition, for convenience of differentiation, a division into stages is provided. When making a diagnosis, this indicator must be indicated, as this gives doctors the most complete picture of the disease.
- 1st stage. The very beginning of the development of the disease. Pathological cells at this time are located only on the surface of the bones and do not penetrate deep into. The size of the tumor reaches only a few millimeters. It is quite simple to get rid of the neoplasm at this time, however, the disease at the 1st stage is extremely rare. The reason is the lack of symptoms.
- 2 stage. Ewing's sarcoma increases in diameter and at the same time begins to penetrate into the deeper layers of bone tissue. The prognosis of treatment in this case is also optimistic. With proper treatment, about 60-65% of patients are cured of pathology.
- 3 stage. Not only bone tissue is involved in the oncological process, but also nearby soft tissues, lymph nodes located nearby. The chances of a successful treatment are slightly lower - about 40%.
- 4 stage. Sarcoma spreads throughout the body and gives secondary foci of cancer. 5-year survival with Ewing's sarcoma and the achievement of remission is possible in 7-10% of cases. With good tumor sensitivity and taking into account bone marrow transplantation at 4 stages, it is possible to increase the patient's chances to 30%.
An important difference between Ewing's sarcoma and many other types of oncology can be called rapid development.
The clinical picture (symptoms)
At the first stages of the development of Ewing's sarcoma, the symptoms practically do not appear, which prevents the early diagnosis of the disease. Moreover, when describing the symptoms, it should be borne in mind: in each case, the symptoms may vary somewhat depending on the location of the tumor.
Pain. Pain can be included in the number of first manifesting symptoms in bone tumors. Initially, pains are characterized as short-term intermittent. During the progression of the disease, they intensify, however, patients often take these symptoms for manifestations of other pathologies. Unlike inflammatory pains, pain with Ewing's sarcoma does not subside at rest and with rigid fixation of the limb. Especially often, patients complain of increased pain at night.
Immobilization of the joint. Approximately 3-4 months after the first manifestation of symptoms, the adjacent joint begins to function poorly. This is manifested in the difficulty of flexion and extension of the limb, the appearance of pain up to complete immobilization.
Redness and pain in soft tissues. A progressive bone tumor gradually involves the soft tissues located in the immediate vicinity. The skin in this area becomes reddened, there is some swelling. During palpation, patients note tissue tenderness.
Enlarged lymph nodes. At stages 1-2 of the sarcoma, only those lymph nodes that are located near the tumor can increase. At stages 3 and 4, an increase and soreness of the cervical, inguinal and other lymph nodes is observed.
General condition worsening. Upon transition to the stage of metastasis, patients experience sharp weight loss, lack of appetite, complete apathy, and rapid fatigue. Perhaps an increase in body temperature, fever.
Characteristic symptoms
There are some symptoms of Ewing's sarcoma, which are characteristic only with the defeat of certain bones:
- bones of the pelvis and lower extremities - the development of lameness;
- spine - spinal cord lesions, radiculopathy, the appearance of delayed reflexes and muscle inhibition;
- chest - blood impurities in sputum, shortness of breath, respiratory failure.
Lab tests
During the diagnostic procedures, doctors pursue several goals at once: to identify the focus of the disease and its nature, and also to check for the presence of metastases in other organs and tissues. For this, several types of laboratory tests and hardware diagnostics are used at once.
Blood analysis. When studying the quantitative composition of the blood, doctors take into account the indicators of red blood cells, platelets and white blood cells. Differences from the norm indicate pathological processes in the body and the spread of the tumor.
Biopsy. By this medical term is meant the extraction of a sample of tissue from a tumor or bone marrow. This procedure is carried out using a long needle. Only after a histological examination of these tissues, doctors can confirm the malignant nature of the tumor.
Hardware Diagnostics
Thanks to hardware diagnostic methods, doctors receive information about the size of the neoplasm, its exact location and the number of foci.
X-ray X-rays are considered one of the most important ways to diagnose bone cancer. If it is necessary to check the condition of the internal bone substance, then an X-ray contrast solution is used. In an x-ray, a healthy bone is gray and a cancerous one is dark.
CT scan. During this procedure, tumor size and the presence (or absence) of metastases are detected.
Magnetic resonance imaging. It acts as an effective diagnostic method.
Chemotherapy treatment
There are several methods of treatment. The choice of this or that approach is based on the stage of the disease, the location of the tumor, concomitant diseases and the age of the patient.
In this case, the treatment of Ewing's sarcoma takes place according to a slightly different scenario than the treatment of other types of cancer. If many other forms of cancer require primarily surgical intervention, then chemotherapy is often prescribed for sarcoma. Carrying out such a course before surgery helps to reduce the size of the tumor.
The principle of chemotherapy is to use very strong drugs. They can be presented in different forms (tablets, injections). Once in the human body, these medicinal substances have a detrimental effect on cancer formation, destroy the structure of its cells and block the development of Ewing's sarcoma. A prognosis with high chances of cure is possible if after chemotherapy no more than 5% of the tumor remains in the patient's body.
Such treatment is carried out in several approaches every 2 or 4 weeks. The most common drugs used in chemotherapy are: vepezid, phosphamide, cyclophosphamide, vincristine, etoposide and some others.
Surgery
Most often, surgical operations are included in complex treatment. After reducing the size of the tumor by chemotherapy, surgeons remove the affected part of the tissue (soft and bone). When large volumes of bone are removed, this area is replaced with grafts. Very rarely, limb amputation (or partial amputation) is required to eliminate the tumor.
If necessary, an additional course of chemotherapy or radiotherapy is prescribed after surgery. This will make it possible to destroy the remains of pathological cells (if any) and thereby prevent the relapse (re-spread) of cancer.
Radiation therapy
Radiation or radiotherapy is another effective way to combat Ewing's sarcoma. During this procedure, the patient's body is exposed to radiation that has a destructive effect on cancer cells. This method of eliminating the pathology is often used in the postoperative period to destroy those cancer cells that remained in the patient's body.
Each of the listed methods of therapy, patients are experiencing quite hard. After surgery, this is a long recovery period, after radiation and radiotherapy there is a period of side effects. This is explained by the fact that radio emission and powerful drugs have a negative effect not only on tumor cells, but also on absolutely healthy tissues (only to a lesser extent). At this time, patients may experience frequent bouts of nausea and vomiting, a sharp drop in immunity, an increased risk of various diseases, and disruption of many body systems.
At the same time, correctly prescribed complex treatment allows you to get rid of even such a terrible and serious disease as Ewing's sarcoma. How many such patients live depends on individual characteristics, however, a positive effect can be achieved even at stage 4 of the disease.