Primary bone cancer is a general term that includes several types of bone sarcoma, which are rare diseases. Among them are osteogenic, chordoma, chondrosarcoma and Ewing's sarcoma. In the structure of the incidence of all malignant bone tumors, the last type occupies 2-3 places. According to various authors, its prevalence in the general structure of oncological pathology of the bone system is 12-20%. It occurs mainly in children and young people. Children aged 10-15 years, who are characterized by intensive growth of the bone system and the beginning of puberty, are most often affected. The disease is most common among boys.
Ewing's sarcoma is characterized by an extremely aggressive course and early metastasis. The period from the beginning of the oncological process to the moment of consulting a doctor and the manifestation of the disease (manifestation of pronounced symptoms) is from 5 to 7 months. Because of this, almost 45% of patients already have micrometastases, and many have metastases in various organs before the exact diagnosis. Lymphogenic spread of tumor cells occurs extremely rarely. As a rule, the process of metastasis to distant organs is carried out through the blood flow system (hematogenous way).
Different bones can be involved in the oncological process, but the most common location of the tumor in the bone skeleton is:
- tibia and tibia;
- pelvic;
- scapulohumeral;
- vertebrae;
- costal bones.
Unlike other types of tumors of the bone system, Ewing's sarcoma in the vast majority of cases affects flat bones. In the case of development in the tubular bones, the disease begins on the diaphysis (in the central part), gradually spreading through the bone canal to the epiphyses (rounded lateral parts).
Factors that contribute to the development of Ewing's sarcoma
There are no reliably confirmed causes and data on the factors that influence the formation of bone tumors. In some cases, the correlation of cancer with traumatic destruction of bone tissue or post-traumatic changes in its structure is revealed.
A number of scientists believe that the basis is a hereditary predisposition caused by various types of gene mutations. In particular, we are talking about the RB1 gene or Paget's disease, as well as some skeletal anomalies (enchondroma, bone aneurysmal cyst). Adverse factors include long-term and high-dose ionizing radiation. However, in the vast majority of patients, there is no connection with predisposing factors.Classification by stages of the disease
The degree of prevalence of the primary tumor formed the basis of classification according to 4 stages:
- The malignant process does not go beyond the bone, it is well differentiated. The size is up to 8 cm, the lymph nodes are not affected.
- The tumor has not yet spread beyond the bone, it is poorly differentiated, the size exceeds 8 cm.
- There are several foci of bone lesions in the primary zone. Highly malignant degree of differentiation (ability to spread).
- The process has gone beyond the bone.
Bone cancer: symptoms and manifestation
Patients complain of pain that occurs periodically in a specific place, the intensity of which increases in the evening or at night and does not stop when the bone is ""unloaded"" or the limb is fixed. In the future, with an increase in the frequency of its occurrence and intensity, sleep is disturbed, and patients are forced to limit physical activity. Hyperemia of adjacent tissues appears in places of bone damage, their swelling, local temperature of the skin rises, subcutaneous veins expand. As the tumor enlarges, a bulky formation begins to be felt, pathological fractures may occur in the places of its growth.
Specific symptoms may occur with one or another localization of the tumor:
- Spinal bone cancer can be accompanied by compression-ischemic myelopathy.
- With tumors in the bones of the chest, respiratory failure is often observed, due to effusion into the pleural cavity (accumulation of fluid) and the presence of blood in sputum.
- If pelvic bone cancer is suspected, symptoms will indicate pelvic organ dysfunction and paralysis.
- Bone tissue tumors of the lower limbs are often accompanied by their asymmetry and lameness.
- When the rib bones are affected, there is a decrease in the mobility of the chest.
The function of the joint adjacent to the affected area is often affected with the formation of a contracture.
As a rule, at the 2nd stage, the symptoms expand due to general manifestations of tumor intoxication: constantly rising temperature, severe weakness, exhaustion. Often there are regional lymphadenitis and anemia, which have a pronounced tendency to increase.
At a late stage of the disease, bone cancer (stage 4), which metastasizes to distant organs and tissues, can affect the central nervous system. This is manifested by various neurological symptoms, depending on the localization of the metastasis in one or another part of the brain.
Diagnostics
To establish an accurate diagnosis, to identify the level of prevalence of the oncological process, a set of diagnostic procedures is used:
- Physical examination.
- X-ray examination of the lesion site in different projections, angiography.
- Ultrasound examination of various systems and organs.
- CT scan of the primary tumor focus. The study makes it possible to determine as precisely as possible the volume of the tumor mass, the presence or absence of its spread along the bone canal, the connection with the tissues surrounding the neoplasia (vessels and nerve fibers).
- Histological examination of biopsy materials from different places.
- If necessary - consultation of specialists of various profiles.
- Blood tests to determine the level of lactate dehydrogenase (LDH). It is an indicator of the level of cell damage, which increases sharply in cancer.
On the basis of a set of data, an accurate diagnosis is made. Some of these methods, in particular CT and MRI, are also used to evaluate the results of one or another method of therapy. The following criteria are used for this:
- The full effect is the regression of the tissue component of the tumor, osteolytic foci and metastases.
- Partial effect — stabilization of the oncological process, reduction of the tumor mass in the amount of 50 percent or more from the initial level.
- Progress of the disease — increase in the size of the lesion, the appearance of new foci, metastases.
Bone cancer: treatment
After a detailed examination, comprehensive therapy is prescribed. The sequence of traditional treatment methods and their intensity are chosen by specialists depending on the stage and characteristics of the oncological process.
Multicomponent polychemotherapy with drugs in various combinations is carried out, taking into account the response of the tumor to the course of treatment. A histologically normal tumor response to a chemotherapy session is a residual number of living tumor cells in the cell, less than 10%.
Ewing's sarcoma is a radiosensitive tumor, so radiation therapy is prescribed to fight it. However, it is more often performed in those patients in whose treatment induction therapy was effective. Irradiation of the focus of the primary bone tumor is carried out after 3-4 courses of chemotherapy.
When neoplasia is detected in the early stages, the tumor is removed, if possible. The scope of the operation is determined on a case-by-case basis. However, it should be understood that radical resection of the focus cannot always be performed, for example, if the bones of the pelvis or spine are affected. However, with the relative availability of neoplasia located in the ribs, clavicle, fibula, scapula, upper limbs, this type of treatment has significant advantages. Advanced surgical techniques allow for gentle surgical interventions that preserve the organ as much as possible. Such a combined approach (situational radical resection of the tumor with intensive methods and dosages of chemoradiation therapy) reduces the likelihood of relapses and the spread of the process to distant organs.
In the presence of metastases in the bone marrow, intensive chemotherapy with combined megadoses of various drugs is carried out, with irradiation of a large area of the body and subsequent operation with bone marrow transplantation from a donor.
Extremely important for the survival of patients are rehabilitation measures (psychological, medical, social). They should be individually oriented, conducted at different stages of patient management: hospital, dispensary. After performing organ-sparing operations, courses of therapeutic gymnastics are conducted in order to restore the mobility of the operated limb.
Bone cancer: how many live?
The prognosis for bone neoplasia depends on many factors:
- places of primary tumor development;
- type of cancer;
- the size of the tumor mass before the treatment;
- presence/absence of metastases;
- level of tumor response to the therapy;
- general condition of the patient's body;
- psychological mood.
Unfavorable factors that worsen the patient's survival prognosis include a high volume of the tumor mass (100 ml or more), damage to the pelvic bones, metastasis to various lymph nodes and bone marrow.
Bone cancer prevention
There are no specific methods of bone neoplasm prevention. As general measures can be recommended:
- Give up smoking and alcohol abuse.
- Lead a physically active lifestyle.
- Maintain immunity: taking herbal preparations that stimulate immunity, hardening water treatments, regular intake of vitamin-mineral complex tablets.
- Follow a rational diet.
- Regularly undergo preventive medical examinations.
However, it should be taken into account that a healthy lifestyle is not a guarantee of reducing the risk of developing any types of malignant diseases. Therefore, if bone cancer is suspected, even minor symptoms should alert you. Seeking specialized professional medical help allows you to start therapy early and increase the effectiveness of treatment measures.
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