Osteogenic sarcoma - Sarcoma, the malignant cells of which originate from bone tissue and produce this tissue. Some of these tumors are dominated by chondroblastic or fibroblastic components. Radiographically divided into osteolytic, osteoplastic (sclerotic) and mixed forms. Osteogenic sarcoma is an extremely malignant neoplasm. Appearing directly from bone elements, it is characterized by a rapid course and a tendency to give metastases early.
The term “osteogenic sarcoma” was introduced in 1920 by James Ewing (Eng.) Russian. .
Osteogenic sarcoma is observed at any age, but about 65% of all cases fall within a period of 10 to 30 years, and most often the development of sarcoma is noted at the end of puberty. Men are affected twice as often as women. Favorite localization is the long tubular bones, no more than one-fifth of all osteogenic sarcomas fall on the proportion of flat and short bones. The bones of the lower extremities are affected 5-6 times more often than the bones of the upper extremities, and 80% of all tumors of the lower extremities nest in the region of the knee joint. The first place in frequency is occupied by the thigh, which accounts for half of all osteogenic sarcomas, followed by tibial bone, humerus, pelvic bones, peroneal, humeral girdle, and ulna. Radial bone, where a giant cell tumor is so often observed, exceptionally rarely gives rise to osteogenic sarcoma. Almost never osteogenic sarcoma comes from the patella. The defeat of the skull occurs mainly in childhood, as well as in old age as a complication of disfiguring osteodystrophy. The typical localization of osteogenic sarcoma in the long tubular bones is the meta-epiphyseal end, and in children and youths, before the onset of synostosis, the bone metaphysis. In the femur, the distal end is usually affected, but about 10% of the osteogenic sarcomas of the thigh nest in the diaphysis and leave the metaphysis intact. In the tibial bone, osteogenic sarcoma is only in one case out of ten located at the distal end - the proximal medial condyle serves as a typical site. The same typical place for the humerus is the area of the roughness of the deltoid muscle.
The onset of the disease is not always accurately determined. Unclear dull pains appear near the joint, as the primary tumor is localized most often near the metaphysial division of the tubular bone. There is pain in the joint without objective signs of effusion in it, often after a past injury. As the boundaries of the tumor expand and the neighboring tissues become involved in the process, the pain increases. A distinct thickening of the metadiaphysis bone appears, pronounced tissue pastosiness, the venous network of the skin is clearly defined. By this time, contracture occurs in the joint, increasing lameness. On palpation - a sharp pain. Severe night pains, not relieved by analgesics and not related to the function of the limb and not subsiding even when fixed in a cast. The tumor rapidly spreads through the neighboring tissues, quickly fills the bone marrow canal, grows into muscles, very early gives extensive hematogenous metastases, especially in the lungs, brain, bone metastases are extremely rare.
Radiodiagnosis of osteogenic sarcomas is simple. Osteoporosis of the bone is characteristic of the initial stages, the contours of the tumor are smeared, it does not extend beyond the limits of the metaphysis. A bone defect is soon outlined. In some tumors, proliferative, osteoblastic processes are noted. In these cases, the detached periosteum is spindle-shaped, swollen, sometimes, interrupting, gives a picture of the "visor". Characteristic, especially in children, is needle periostitis, when osteoblasts produce bone tissue along the blood vessels, that is, perpendicular to the cortical layer, forming the so-called spicules. The differential diagnosis of osteogenic sarcoma is carried out between chondrosarcoma, eosinophilic granuloma, cartilaginous exostoses, osteoblastoclastoma.
Treatment of osteogenic sarcoma includes the following stages:
- Preoperative chemotherapy to suppress micrometastases in the lungs, reduce the size of the primary tumor site and evaluate the histological response of the tumor to chemotherapy, which determines the further method of treatment. The following drugs are currently used for the treatment of osteogenic sarcoma: high-dose methotrexate, adriblastin, ifosfamide, platinum preparations, (carboplatin, cisplatin), etoposide.
- Mandatory operation. If previously resorted to extensive surgery, often involving the amputation of the entire limb, it is currently limited to a sparing operation. In this case, only parts of the bone are removed and replaced with an implant made of plastic, metal, or cadaveric bone. The organ-preserving surgery is refused in cases where the tumor invades the neurovascular bundle, if a pathological fracture occurs, as well as for large sizes of the tumor and its germination in soft tissues. The presence of metastases is not a contraindication to conserving surgery. Large lung metastases are also surgically removed.
- Postoperative chemotherapy based on the results of preoperative chemotherapy.
Radiation therapy for treatment is ineffective due to the fact that the cells of osteogenic sarcoma are insensitive to ionizing radiation. Radiation therapy is carried out if, for any reason, surgery is not possible.
The emergence of new approaches that provide for adjuvant and neoadjuvant chemotherapy and radiotherapy in addition to surgical interventions and the development of benign methods significantly increase the survival of patients with osteogenic sarcoma. Significantly increased the chances of treatment of patients with pulmonary metastases.
Radical surgery with preservation of the limb (possibly in more than 80% of patients), together with preoperative and postoperative chemotherapy, gives the best results. When treating patients with localized osteogenic sarcoma, 5-year survival exceeds 70%. The survival rate of patients with tumors sensitive to chemotherapy varies between 80-90%.
What contributes to the development of sarcoma?
The causes of the appearance of malignant neoplasms of bones were not found. However, several provoking factors were identified, including chemotherapy and radiation used in the treatment of malignant tumors of other organs. Fracture or contusion does not have a direct effect on the formation of sarcoma. However, intense pain or the appearance of an unusual seal after the injury caused a person to consult a doctor, who discovers a malignant neoplasm.
In rare cases, osteosarcoma forms on the background of a long course of osteomyelitis. The risk of developing cancer increases the Paget's disease - a deforming inflammatory process. Most sarcomas form in the lower femur, affecting the metaphysis, the part of the bone that lies between the diaphysis and the articular surface.
How does femoral sarcoma manifest?
In the early stages, osteosarcoma develops asymptomatically. Then there are aching pains in the area of the joint. By nature, they may resemble signs of rheumatoid arthritis, myalgia or periarthritis. The joint with sarcoma does not fill with fluid. The size of the neoplasm increases, it captures the soft tissues, the pain syndrome becomes more pronounced. Part of the bone increases in size. The affected area swells, spider veins appear on the skin. A contracture is formed in the nearby joint. On palpation, pain increases. A characteristic sign of hip sarcoma is lameness.
At the next stage, the pain becomes so strong that it can be impossible to remove it with standard painkillers. They torment a person both during physical activity and during rest. The tumor continues to grow, affecting the muscles and bone marrow. Metastasizes sarcoma by hematogenous, cancer cells spread throughout the body, settling in the liver, lungs and brain. When specifying the diagnosis, it is obligatory to assign a cancer to one or another stage. Osteogenic sarcoma of the femur is localized (cancer cells do not extend beyond the affected part of the bone) and metastatic (secondary foci are found in other tissues). Some experts believe that osteosarcoma may have micrometastases that cannot be detected when performing diagnostic procedures.
Separately, the multifocal form, simultaneously affecting several bones, is considered. Like all malignant tumors, sarcoma may recur within a few months after therapy. The neoplasm can develop both at the site of the distant and other organs. Most often, relapse occurs within the first 2 years after completion of the course of treatment; a tumor forms in the lungs. Over time, the likelihood of recurrence of the disease decreases.
Diagnosis and treatment of the disease
Examination of the patient begins with a primary examination, laboratory and hardware diagnostic procedures. When interviewing a patient, the physician should pay attention to the gradual development of symptoms of the disease and aching pain in the joint, increasing with time. Edema of the affected area in the first stage is extremely rare. Vascular dilatation is also a manifestation of the late stages of the development of sarcoma. Even if these symptoms are absent, the doctor does not exclude the presence of sarcoma. The final diagnosis is made on the basis of the results of x-ray examination of the bone and histological analysis of the tumor tissues. To detect secondary foci in other organs allow CT and MRI.
In the early stages revealed a decrease in bone density and fuzzy boundaries of the tumor. Subsequently, bone destruction occurs. In children, needle-like osteophytes are found - bone growths that are perpendicular to the surface. A sample taken for histological examination contains altered cells of the connective and bone tissue, as well as osteoid inclusions.
The treatment is selected depending on the stage of the cancer, the prevalence of the malignant tumor, the age of the diseased, the general condition of the body. Therapy usually proceeds in 3 stages. Preliminary chemotherapy helps to reduce the tumor in size, the destruction of individual metastases. The next step is the operation. In the past, osteosarcomas were treated by amputation of limbs. Currently, such a surgical procedure is being replaced by resection of the affected bone with further prosthetics. Amputation is prescribed for the detection of large tumors, pathological fractures, the spread of cancer cells to muscle tissue and nerve endings.
Not only the primary focus, but also large metastases in other organs are surgically removed. It is obligatory to excise the regional lymph nodes. Postoperative chemotherapy can destroy the remaining tumor cells and prevent the spread of metastases. Irradiation in osteosarcoma has low efficiency and is carried out when it is impossible to perform the operation.
The prognosis for malignant bone tumors is determined by many factors: the size of the neoplasm, the stage of its development, the presence of metastases, the general condition of the body. The chances of recovery depend on the timeliness of chemotherapy and surgery. A more favorable prognosis becomes subject to the complete elimination of the tumor and reduction of metastases after a course of chemotherapy. Previously, osteosarcoma was considered the most aggressive malignant neoplasm, even amputation did not save the patient's life. Currently, there are chances for recovery even when lung metastases are detected.
What causes can cause osteosarcoma
Doctors say that in the development of osteogenic pathological formation, there is a relationship with bone growth. therefore with the rapid growth of bones, there is a possibility of osteosarcoma.
When a tumor is detected in children, as a rule, their growth is higher than that of their peers. Sarcoma cancer cells affect those parts of the skeleton that grow most quickly.
But this certainly does not mean that all tall children necessarily suffer from osteogenic sarcoma. They only increase by a few percent the risk of falling into the group of patients with osteosarkma.
Other indirect (direct, as with other types of cancer, science is not known) reasons that may affect the development of osteogenic sarcoma include:
- The impact on the body of ionizing radiation. A tumor may appear 4 to 40 years after receiving a radiation dose.
- Bone injury: fractures, bruises. After that, a bone tumor may begin to develop.
- Paget's Disease. In patients with this diagnosis in 2% of cases, bone damage by osteosarcoma is possible.
- Benign tumors bones can also develop into malignant sarcoma.
- After cure oretinoblastoma, 50% of patients may have a relapse in the form of osteogenic sarcoma. These two diseases have changes in 13 pairs of chromosomes.
What symptoms describe the course of the disease?
Osteogenic sarcoma is manifested by the following symptoms:
- Dull pain in the area of bone damage by cancer cells
- Painful sensations are permanent, with increasing strength (very often the pain intensifies at night)
- Affected area increases in volume
- If a tumor affects the extremities, then due to the swelling at the site of the tumor, the mobility and functions of the extremity are disturbed.
Osteogenic sarcoma aggressive tumor therefore, its metastases quickly affect other organs of the body. In 80% of cases when treating patients, they already have metastases that can be detected by computed tomography. In 20% of metastases can be detected even with the help of a conventional X-ray machine.
The bones do not have the lymphatic system, therefore metastases in regional lymph nodes are extremely rare. But if they are still there, then the prognosis for survival - this circumstance reduces.
The patient has a feeling that the pain emanates from the joints, and many begin to treat them on their own. At this time, the tumor continues to grow and the pain only intensifies.
A noticeable condensation begins to appear in the affected area, which most likely resembles edema. Above the seal, the venous mesh is clearly visible on the skin.
In addition, the patient appears new symptom - lameness . Pain at night can not be reduced by taking painkillers. When you feel the tumor felt severe pain.
For this period the tumor metastasizes to the brain and lungs.
What is the prognosis for patients?
More than 80% of patients may have organ-sparing operations.
Modern methods of sparing chemotherapy and radiotherapy, in conjunction with surgical intervention can significantly increase the survival of patients with this diagnosis.
The prognosis for 5-year patient survival depends on the volume of the tumor.
Types of sarcoma
Doctors distinguish about 3 types of this disease:
Each of these species has characteristic features that are unique to them.
In osteolytic sarcoma, a person develops 1 site of bone disorder. It has blurred, irregular contours. It is noteworthy that the process of destruction is rapidly spreading and the focus may increase in size several times. Also, this stage is characterized by the appearance of disorders in soft tissues and the appearance of metastases in nearby organs.
Osteoplastic sarcoma is characterized by rapid tumor development. This process occurs several times faster compared with a bone disorder. As a result, needle and fan-shaped growths are formed. Due to the active development of the cancer process in humans, irreversible morphological disorders occur in problem areas of the body and even the face.
In a mixed form of sarcoma, the patient develops a process of destruction together with the formation of pathogenic bone structures in various proportions.
Doctors say that it is impossible to accurately determine the main cause of sarcoma. However, they identify several factors that may lead to the development of this problem:
- the presence of various injuries
- development of genetic mutations. They usually lead to the appearance of sarcoma of the skull in newborn babies,
- hereditary retinal tumor,
- in Li-Fraumeni syndrome or mutations in the p53 gene,
- with Paget's disease and other benign bone pathologies,
- in the presence of anomalies in the development of bones of the skeleton, this is especially evident at the time of puberty,
- with frequent radiation exposure, especially in the treatment of other oncological diseases.
Osteogenic sarcoma usually appears in citizens who are exposed to these symptoms.
Symptoms of osteosarcoma
The initial stage of the disease does not show any typical signs. As a result, it is almost impossible to identify it. Osteogenic severe sarcoma is characterized by the following symptoms:
- dull pain localized near the joint, in which there is a focus of the disease. It is noteworthy that the thigh hurts exactly at night. Doctors say that these pains only increase with time and it is impossible to get rid of them with the help of potent drugs,
- the presence of pathologies in nearby organs,
- thickening and disturbances in the bones of the problem area,
- the appearance of edema in the bones,
- the presence of pasty tissues: bone tissue is similar to dough or soft clay,
- appearance of a venous network on the skin surface,
- restriction of movement in the joint,
- the development of lameness
- the appearance of pain during the feeling of the problem area,
- the spread of the tumor to nearby tissues and filling the bone marrow with the oncological process,
- defeat oncology of muscle tissue
- the appearance of metastases in the lungs, brain and liver.
The initial stage of osteosarcoma can last for 3 months. The development of this disease in young children is characterized by the appearance of deformities in the bone tissue. This is manifested by the development of acicular periostitis. Osteosarcoma is characterized by the appearance of osteoblasts that form spicules along the blood vessels.
Treatment of osteogenic sarcoma
To date, only radical methods have been used to treat this dangerous disease. These include complete removal of the affected limb.
Now for the treatment of osteosarcoma, the following methods are used:
- Chemotherapy. It can be used both before surgery and after it. Typically, patients are given such drugs as: Methotrexate, Doxorubicin, Cisplastin, Carboplatin, Cyclophosphamide. This method of treatment is aimed at reducing the spread of cancer cells. However, a significant disadvantage of this therapy is that it also negatively affects healthy cells of the body.
- Surgical intervention. This method aims to remove the focus of the disease and preserve the affected limb. However, by no means in all cases during the operation specialists can maintain a limb. Full removal of the limb is carried out only in cases where the cancer process has spread to nearby tissues, as well as when there is germination in the nerve fibers and blood vessels.
All treatment is carried out only under the supervision of experienced doctors and in the hospital. Installing a prosthesis to a patient allows you to return the lost motor activity already after 6 months after surgery. Doctors say that surgery should not be used in case of sarcoma in the skull, spinal column and pelvic bones.
It is noteworthy that when osteogenic sarcoma of the hip appears, radiotherapy methods are not used. They can be applied only in the case when it is necessary to get rid of a part of the neoplasm or to eliminate existing relapses.
Survival prognosis for osteogenic sarcoma
Many patients who have been diagnosed with osteogenic sarcoma often wonder: "How long do people live with this disease?". Doctors say that with early diagnosis and well-conducted treatment, the survival rate is about 70%. Thus, patients can live for about 5 years.
If a patient has tumor tumors that are sensitive to the chemotherapy and radiation exposure, the survival rate is about 90%. However, if a patient has metastases, doctors do not give a very favorable prognosis. The survival rate in this case is from 10 to 15%.
What causes osteogenic sarcoma?
Most cases of the disease are spontaneous, however, the importance of radiation exposure and previous chemotherapy in the subsequent development of osgeogenic sarcoma, as well as the connection with the presence of the reticoblastoma RB-1 gene, has been proven. Increased incidence is recorded in patients with Paget's disease (deforming osteosis) and Oleo (dyschondroplasia).
A typical source of tumor growth is the metadiaphysical growth zone. The defeat of the metadiaphysis for osteogenic sarcoma is more characteristic than for Ewing, but cannot serve as a pathognomonic symptom. In 70% of cases, the zone adjacent to the knee joint — the distal metadiaphysis of the femur and the proximal metadiaphysis of the tibia — is affected. In 20% of cases, the proximal metadiaphysis of the humerus suffers. The defeat of the bones of the spine, skull and pelvis occurs very rarely and is usually associated with a fatal prognosis due to the impossibility of a radical operation.
How does osteogenic sarcoma manifest?
Osteogenic bone sarcoma, which usually occurs in the central regions, in the process of growth destroys the cortical layer and draws the soft tissues of the limb into the tumor. For this reason, at the time of diagnosis, it usually acquires a two-component structure, i.e. consists of bone and extra bone (soft tissue) components. A longitudinal incision of the bone resected together with the tumor reveals a wide invasion of the bone-marrow canal.
According to the morphology of tumor growth, the following types of osgeogenic sarcoma are distinguished: osteoplastic (32%), osteolytic (22%) and mixed (46%). These morphological types correspond to the variants of the X-ray picture of the tumor. Survival of patients does not depend on the X-ray morphological variant. Separately, the disease with the cartilage (chondrogec) component is isolated. Its frequency is 10-20%. The prognosis for this variant of the tumor is worse than with osteogenic sarcoma with the absence of the cartilage component.
According to the location relative to the bone marrow canal, the classical, periosteal and parostal forms of the tumor are radiologically separated.
- Classical osteogenic sarcoma accounts for the vast majority of cases of this disease. In her case, typical clinical and radiological signs of damage to the entire bone diameter are noted with the process coming out to the periosteum and further into the surrounding tissues with the formation of a soft-tissue component and filling the tumor masses of the medullary canal.
- Osteogenic periosteal sarcoma is characterized by a lesion of the cortical layer without spreading the tumor to the medullary canal. Perhaps this form should be considered an option or a stage in the development of a classical tumor until it spreads to the bone marrow canal.
- Parosteal osteogenic sarcoma emanates from the cortical layer and spreads along the periphery of the bone, without penetrating deep into the cortical layer and into the medullary canal. The prognosis for this form, constituting 4% of all diseases and occurring predominantly in older age, is better than for the classical form.
How is osteogenic sarcoma treated?
The treatment strategy from the description of this Ewing tumor in 1920 to the 70s. XX century. was reduced to organ-replacing operations - amputations and disarticulations. According to the literature, the 5-year survival rate with this tactic did not exceed 20%, and the frequency of local relapses reached 60%. Death of patients occurred, as a rule, within 2 years as a result of metastatic lung damage. From modern positions, taking into account the ability of osteogenic sarcoma to early hematogenous metastasis. the possibility of a favorable outcome without chemotherapy is unlikely.
In the early 70s. XX century. The first cases of effective chemotherapeutic treatment of this tumor have been reported. Since then, the position has been firmly established that, as with other malignant tumors in children, with osteogenic sarcoma, only treatment including polychemotherapy will be successful. Currently, the treatment proved the effectiveness of anthracyclic antibiotics (doxorubicin), alkylating agents (cyclophosphamide, ifosfamide). platinum derivatives (cisplatan. carboplatin), topoisomerase inhibitors (etoposide) and high doses of methotrexate. Chemotherapy is carried out in nsoadjuvant (before surgery) and adjuvant (after surgery) regimens. Regional (intra-arterial) chemotherapy is not accompanied by higher efficacy compared with systemic (intravenous) chemotherapy. Given this, one should prefer the intravenous method of infusion of cytostatics. Osteogenic sarcoma refers to radioresistant tumors, radiation therapy is not used with it.
The question of the sequence of the surgical and chemotherapeutic stages of treatment of pulmonary metastases of osteogenic sarcoma is decided depending on the severity and dynamics of the metastatic process. In cases where up to four metastases have been identified in one lung, treatment is advisable to begin with the removal of metastases followed by chemotherapy. If more than four metastases are detected in a light or bilateral metastatic lesion, chemotherapy is initially performed followed by a possible radical surgery. With the initially unresectable nature of lung metastatic lesions, the presence of tumor pleurisy and the preservation of the unresectable metastases after chemotherapy treatment, the attempt at surgical treatment of metastases is not advisable.
What is the prognosis of osteogenic sarcoma?
The prognosis is better in the age group from 15 years to 21 years compared with younger patients; in all age groups, the prognosis is better for females. Osteogenic sarcoma has a worse prognosis, the larger the bone in which it originated, which is associated with a possible longer latent course of the tumor process and the achievement of a greater mass of tumor cells before the stage of diagnosis and the start of special treatment.
Currently, 70% of the total 5-year survival rate for osgeogenic sarcoma is achieved, subject to programmed treatment. Survival rates are not different depending on organ-carrying or conserving surgery. In metastatic lung disease, the overall 5-year survival rate is 30-35%. The early appearance (up to 1 year from the end of treatment) of lung metastases more likely reduces the likelihood of recovery, compared with the diagnosis of metastases at a later time. The prognosis is fatal with unresectable pulmonary metastases, metastatic lesions of bones and lymph nodes, and inadequate primary tumor. The prognosis is worse, the younger the patient. Girls recover more often than boys.
General information about the disease
This disease has an extreme malignancy, characterized by a rapid course and the ability to quickly give metastases.
Bone cancer can develop in people of any age, but most often occurs during puberty in children and adolescents between the ages of 10 and 20, as well as in very elderly people. Typically, the pathology is observed in men, they suffer from this disease two times more often than women. The favorite location of the tumor - cylindrical or triangular bones, so the lower limbs are damaged more often than the upper. A large percentage of tumors occur in the area of the knee joint, but the tumor has a high percentage of metastasis. Outbreaks can be observed in various internal organs, especially in the lungs and liver.
There is an opinion that bone cancer develops due to long-term inflammatory processes, for example, chronic osteomyelitis.
Therefore, the following bones are most often affected:
Radial bone and patella are rarely affected by osteosarcoma. The defeat of the skull occurs most often in childhood, as well as in old age as a result of complicated osteodystrophy.
Causes of the disease
This type of tumor has some connection with the rapid growth of bones. Therefore, osteosarcoma affects mainly children of high growth, whose height is above the age norm limits. In other words, the disease develops in very tall children, since osteosarcoma affects the rapidly growing parts of the musculoskeletal system.
One of the reasons for the development of this disease may be radiation, which for some reason affects children and adolescents. This fact can be a cause of hormonal disorders and weaken the child's body. However, children or teenagers do not get sick right away. The gap between the effects of radiation and the development of the disease can be on average about 10 to 15 years.
The development of the disease is observed in individuals who have benign tumors (osteochondromas) in history.
The development of bone tumors can occur as a result of injury. It turns out that the injury attracts the attention of a specialist and forces to conduct instrumental examinations (X-rays and MRI), during which the disease is diagnosed. Paget's disease can be the starting point of osteosarcoma, often with multiple bone lesions.
Osteosarcoma is observed in individuals who have previously had been ill with retinoblastoma (a tumor with a hereditary etiology). At the same time, 50% of secondary tumors already occur in osteosarcoma, and in both diseases genetic changes are observed in the chromosomes.
Diagnosis of osteosarcoma
Diagnosis is carried out with the help of regenerative research. Osteoporosis of the bone is first diagnosed, since there are no clear contours of the tumors, they are blurred. Over time, the bone begins to break down, there are metastasis processes.
For the correct diagnosis, conduct a series of additional studies:
- morphological examination of the tumor (biopsy, knife biopsy, trephine biopsy),
- osteoscintigraphy (OSG),
- computed tomography (CT),
- magnetic resonance imaging (MRI),
- angiography (vascular examination).
Types of osteogenic sarcoma
Osteosarcoma has the following varieties:
- small cell,
- osteosarcoma of the pelvis.
These are rare types of bone cancer, each with its own symptoms and origins.
Teleangiectatic - in the picture it resembles an aneurysmal bone cyst and giant cell tumor. The course of the disease is no different from the classical variants of osteosarcoma.
Juxtacortical - develops from the cortical layer of the bone, and tumor tissue surrounds the bones from all sides, but usually does not penetrate the medullary canal. This type of osteosarcoma has a low threshold of malignancy, it proceeds slowly and practically does not metastasize. However, treatment should be carried out in a timely manner, since this tumor is able to progress, and changes the degree of malignancy to a higher one.
Periosalnaya - localized on the surface of the bone, has a soft structure, also does not penetrate into the bone marrow canal.
Intraosal The tumor has a low degree of malignancy and is regarded as a benign tumor. However, in the absence of adequate treatment, they are also prone to relapse into a more malignant variant.
Multifocal - characterized by multiple foci in the bones, which are similar to each other. It is still not known whether they all occur at once or whether there is rapid metastasis of one lesion. The prognosis is unfavorable.
Extraskeletal - rare tumor, which is manifested by the defeat of other areas:
The prognosis of the disease is extremely unfavorable. The tumor is very aggressive and almost impossible to chemotherapy.
Small cell tumor has a high degree of malignancy. In its morphology, it differs from all other species. Most often affects the femur bone.
Osteosarcoma of the pelvis is characterized by an extremely poor prognosis. The tumor progresses rapidly and widely spreads to neighboring tissues, because, because of its anatomical structure, it does not encounter fascial barriers in its path.
The following types of osteosarcoma are also divided:
Osteolytic sarcoma is manifested by the presence of a single lesion that has fuzzy boundaries. The destruction of the bone progresses rapidly, increasing in length and width. Soft tissue is damaged, then metastases penetrate into other organs.
The osteoplastic form leads to the rapid growth of a neoplasm, which leads to the destruction of bone tissue. The tumor quickly fills the voids in the spongy substance, forming needle growths.
Mixed form involves a combination of symptoms of the above two types of sarcoma.
The treatment of the disease is carried out in three stages:
First stage. Chemotherapy effectively neutralizes dividing cancer cells, but unfortunately causes damage to healthy cells too. Preoperative chemotherapy is performed to suppress lung metastases. For this purpose, the following drugs are used:
- Methotresat (in combination with Leucovorin),
Second phase. Surgical treatment is performed for excision of the tumor, which allows you to save a significant part of the bone. In the past, the patient was completely removed the limb, but the modern development of medicine allows excision only of the affected part of the bone. The removed area is replaced with a metal, plastic, or cadaveric bone implant. Large metastases in the lungs are also excised by surgery. The surgical method of treatment cannot be applied when a tumor is localized: in the spine, in the bones of the pelvis, in the cranial bones.
The third stage. Postoperative chemotherapy. The purpose of this treatment is to consolidate the result.
Radiation therapy in the case of osteogenic sarcoma is ineffective because the tumor cells are insensitive to ionizing radiation. Radiation therapy is used when there are contraindications for surgical treatment.
In severe cases, you still have to remove the affected limb. This happens when the tumor grows into neuro-vascular tissue and is of enormous size.
The use of modern therapies, which include various types of chemotherapy and surgery, give very good results. This contributes to the survival rate of patients with this diagnosis.
Osteosarcoma of the femur
Currently, the exact causes of this disease are not fully known. However, there are several factors that can trigger the development of pathology. These include contacts:
- with carcinogens,
- ionizing radiation
Osteosarcoma often develops as a result of injury. However, this is not a cause, but a catalyst for the occurrence of a tumor.
The main cause of femoral sarcoma is the rapid growth of the skeleton during the pubertal period. Another reason are genetic abnormalities, in particular the mutation of certain genes.
At the initial stage of development, the disease does not manifest itself. Then, when the tumor grows, pain appears, limb functions are disturbed, and swelling appears. As the pathological process progresses, all new tissues and joints are affected, and the bones grow into the bone marrow. Malignant cells spread with blood throughout the body, forming metastases in the internal organs.
The treatment of ailment includes:
- preoperative therapy
- postoperative therapy.
Preoperative therapy is performed to reduce the tumor. To do this, use drugs from the group of cytostatics.
Surgical treatment involves radical amputation of the affected limb. However, at present, femoral cancer is treated more gently. The organ is preserved by removing the pathological formation, and the implant is inserted instead.
Postoperative chemotherapy is carried out to destroy the remaining malignant cells, as well as to remove foci of metastasis. Unfortunately, even this treatment does not always provide complete relief from bone cancer. Osteosarcoma can recur after 2 to 3 years and symptoms reappear.
Ewing's sarcoma in children
This type of bone cancer is found predominantly in children and is most often found in bones, less often it can develop in soft tissues: connective, muscle, adipose and peripheral nerve tissues. Ewing's sarcoma is extremely aggressive and metastasizes so quickly that without adequate and quick treatment, this disease is fatal.
This form of bone cancer is quite rare. It affects mainly children and adolescents, but it is also found in very elderly people. Boys and men get sick much more often than girls. More than half of all cases are children and adolescents of the age range from 10 to 20 years.
Ewing's sarcoma can infect any bone. Most often, a tumor is detected in the pelvic bones; it can also appear in the long tubular bones of the thigh, tibia and ribs. The tumor can spread both inside the bone and in the soft tissues around the bone. Sometimes it can immediately grow in the soft tissues around the bone, and in the bone itself it may not be. In this case, we are talking about Ewing's extracellular sarcoma. A cancerous tumor very often gives metastases, and they already exist when they first go to the doctor. Metastases are most often formed in the lungs, in the bones and in the bone marrow.
Prediction and prevention of the disease
The forecast depends on several factors:
- the size and location of the neoplasm
- stage of the disease (tumor localization, the presence of metastases in other organs),
- age and general health of the patient.
Postoperative prognosis depends on the effectiveness of preoperative chemotherapy and the result of the operation. The most favorable prognosis is a significant reduction in metastases after chemotherapy, as well as with complete removal of the tumor.
In the past, osteosarcoma was positioned as a serious malignant disease with a very unfavorable outcome (even with the removal of a limb, the survival rate was no more than 5–10%). At present, thanks to new methods of treatment and effective organ-preserving surgical interventions, the chances of a safe prognosis have increased significantly, even if there are metastases in other organs. Thus, with localized osteosarcoma, the survival rate is 70%, and with osteogenic sarcoma, which is sensitive to chemotherapy, the survival rate is over 80 - 90%.
As for prevention, there are currently no effective preventive measures against this disease. Experts advise to timely treat injuries and damage to the joints and bones. People with a genetic predisposition to the disease is recommended to be regularly monitored by a doctor to identify the first signs of bone tissue degeneration.
Preventing osteogenic sarcoma in humans
Doctors say that currently there are practically no effective ways to prevent the development of osteogenic sarcoma. Experts note that in the presence of injuries and injuries in the joints and bones, they must be treated. In the event that a person is prone to the appearance of genetic abnormalities, he must always be under the control of a specialist and undergo a complete examination of the whole organism.
General characteristics of the disease
Osteogenic sarcoma is an extremely malignant neoplasm. Appearing directly from bone elements, it is characterized by a rapid course and a tendency to give metastases early.
Previously, the prognosis for this disease was very unfavorable. Five-year survival was observed in less than 10% of cases. Currently, due to the emergence of new methods of treatment, the chances of patients have increased significantly. Today, more than 70% of patients with localized osteosarcoma and 80-90% with a tumor-sensitive chemotherapy experience 5 years after diagnosis.
Osteosarcoma is more common in younger patients. More than two thirds of all cases of the disease occur at the age of 10-40 years. In this case, men suffer twice as often as women. The peak of the incidence occurs during the period of rapid growth: 10-14 years for girls and 15-19 years for boys. After 50 years, such a tumor is extremely rare.
It is assumed that the appearance of bone cancer is associated with intensive development of the body in childhood and adolescence. The risk group includes, above all, children, whose height is above average. Also, according to statistics, boys are subject to this disease to a much greater extent than girls. Osteosarcoma of bone is often solitary in nature, i.e. It affects both soft tissues directly around the localization of a neoplasm, and the entire body through metastasis.
Causes and predisposing factors
The causes of the disease are not yet clear. However, there are a number of circumstances in which the risk of its occurrence increases. These include radiation therapy and chemotherapy, conducted earlier for another cancer.
In itself, injury does not affect the likelihood of a tumor. However, in some cases, it is a fairly serious traumatic injury (pathological fracture) or unusual pain persisting for a long time after the injury in the affected area causes the patient to go to the traumatologist and subsequent detection of osteosarcoma.
In some cases, osteosarcoma develops on the background of chronic osteomyelitis. The risk of tumor formation also increases with Paget's disease (deforming osteitis).
The disease usually affects the long tubular bones. The proportion of short and flat bones is no more than 20% of the total number of osteosarcomas. Lower limbs are affected 5-6 times more often than the upper. Moreover, about 80% of the total number of osteogenic sarcomas develops in the region of the distal end of the femur. Further, in descending order of frequency, tibial, brachial, pelvic and fibula bones are located. Then - the bones of the shoulder girdle and ulna. The bones of the skull are rarely affected, mostly in children or in the elderly.
A typical localization of osteosarcoma is the region of the metaphysis (the part of the bone located between the articular end and the diaphysis). However, about 10% of the total number of osteosarcomas of the hip are found in its diaphyseal part, while the metaphysis remains intact.
In addition, osteosarcoma has “favorite” locations in each individual bone. So, at the hip the distal end is usually affected, at the tibia - the internal condyle, at the humeral - the area where the deltoid muscle is located. Stay close to us on facebook:
The disease begins gradually, gradually. At first, the patient is bothered by dull obscure pains that occur near the joint. By their nature, they may resemble pain in rheumatic myalgia, arthralgia, myositis, or periarthritis. At the same time, effusion (fluid) in the joint is absent.
The tumor gradually grows, adjacent tissues are involved in the process, and the pain intensifies. The affected division of the bone increases in volume, the soft tissues above it become swollen, phlebectasia (a network of small dilated veins) occurs on the skin. Formed contracture in the adjacent joint. With the localization of osteosarcoma on the bones of the lower limb develops lameness. Palpation of the affected area is sharply painful.
After some time, the pain becomes unbearable, depriving sleep. They appear both during exercise and at rest, and are not removed by conventional analgesics. Osteosarcoma continues to grow rapidly, spreading to neighboring tissues, germinating muscles and filling the bone marrow channel. The tumor cells spread through the bloodstream, "settling" in various organs and forming metastases. In this case, the brain and lungs are most often affected.
Rare variants of osteosarcoma.
Teleangiectatic - radiologically resembles an aneurysmal bone cyst and giant cell tumor, manifested by the presence of lytic foci with mild sclerosis. The course of the disease and the response to chemotherapy is almost the same as the standard osteosarcoma variants.
Juxtacortical (paraosalnaya) - comes from the cortical layer of the bone, the tumor tissue can surround the bone from all sides, but as a rule, does not penetrate into the bone marrow canal. The soft-tissue component is absent; therefore, it is difficult to distinguish a tumor from an osteoid radiologically. As a rule, this tumor of low degree of malignancy, flows slowly, almost does not metastasize. However, paraosal osteosarcoma requires adequate surgical treatment, almost the same as the standard tumor variants. Otherwise, this tumor recurs and at the same time the tumor component changes the degree of malignancy to a higher one, which determines the prognosis of the disease.
Periosalnaya - as well as paraosal, it is located on the surface of the bone and has a similar flow. The tumor has a soft-tissue component, but does not penetrate into the medullary canal.
Intraossal tumors with a low degree of malignancy, well differentiated, with minimal cellular atypia, can be regarded as a benign tumor. But they also tend to recur locally, with the tumor component changing to a more malignant variant.
Multifocal - It manifests itself in the form of multiple lesions in the bones, similar to each other. It is not clear until the end whether they appear immediately or whether there is rapid metastasis from one nidus. The prognosis of the disease is fatal.
Extraskeletal osteosarcoma- a rare malignant tumor, characterized by the production of osteoid or bone tissue, sometimes with cartilage tissue - in the soft tissues, most often, of the lower extremities. But there are also lesions in other areas, such as the larynx, kidneys, esophagus, intestines, liver, heart, bladder, etc. The disease prognosis remains poor, sensitivity to chemotherapy is very low. The diagnosis of extraskeletal osteosarcoma can only be established after excluding the presence of bone tumor foci.
Small cell osteosarcoma - a highly malignant tumor, in its morphological structure, differs from other variants, which determines its name. Most often this tumor is localized in the femur. Small cell osteosarcoma (differential diagnosis with other small cell tumors) necessarily produces osteoid.
Osteosarcoma of the pelvis - despite the improvement in overall survival for osteosarcoma, it has a very poor prognosis. The tumor is characterized by a rapid and wide spread in the tissue and along them, since, due to the anatomical structure of the pelvis, it does not encounter significant fascial and anatomical barriers in its path.
When making a diagnosis of osteosarcoma staging is carried out - assessment of the prevalence of the process. Given the prevalence, all osteosarcomas are divided into two large groups:
- Localized sarcoma. Tumor cells are only in the place of its initial localization or surrounding tissues.
- Metastatic sarcoma. A metastatic lesion of other organs is detected.
It is assumed that in 80% of patients there are micrometastases that are so small that they are not detected during additional studies.
In addition, they produce multifocal sarcoma, in which tumors simultaneously appear in several (two or more) bones.
Osteosarcoma, like other malignant tumors, in some cases returns after treatment. A new tumor may occur both at the site of the previous one and in another part of the body (in the area of metastases). This condition is called relapse. Relapse with osteosarcoma most often develops 2-3 years after treatment and is found in the lungs. Further relapses are also possible, but the probability of their development decreases. Stay close to us on facebook:
Course of the disease
The prognosis for osteosarcoma depends on certain factors both before and after treatment. Factors affecting the prognosis prior to the treatment of osteosarcoma:
- The size and location of the tumor.
- Stage osteosarcoma (localized tumor, the presence of metastases in other organs).
- Age and general health of the patient.
After treatment, the prognosis is determined by the effectiveness of chemotherapy and the result of the operation. A more optimistic prognosis is with a significant decrease in mestostates and primary lesion after chemotherapy, as well as with complete removal of the tumor.
In the past, osteosarcoma was among the malignant tumors with the most unfavorable prognosis. Even though during the treatment, severe mutilated operations were performed - amputations and exarticulation of the extremities, the five-year survival rate did not exceed 5-10%.
Currently, due to the emergence of new methods of treatment and the use of effective organ-preserving operations, the chances of patients at a favorable outcome have increased significantly, even in the presence of lung metastases. Five-year survival with localized osteosarcoma today is more than 70%, and in the group with osteosarcomas sensitive to chemotherapy, this figure is even higher - 80-90%. Stay close to us on facebook: