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Osteogenic sarcoma
Last reviewed: 23.04.2024
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Osteogenic sarcoma is a malignant bone tumor that develops as a result of malignant transformation of rapidly proliferating osteoblasts and consists of spindle-shaped cells forming a malignant osteoid.
ICD-10 code
- C40. Malignant neoplasm of bones and articular cartilage of extremities.
- C41. Malignant neoplasm of bones and articular cartilages of other and unspecified sites.
[What causes osteogenic sarcoma?
Most cases of the disease are spontaneous, but the significance of radiation exposure and previous chemotherapy in the subsequent development of osseogenic sarcoma, as well as the connection with the presence of the RB-1 retikoblastoma gene, is proved. An increased incidence is recorded in persons with Paget's disease (deforming ostosis) and Olia (dyskondroplasia).
A typical source of tumor growth is the metadiaphyseal growth zone. The defeat of the meta-diaphysis for osteogenic sarcoma is more characteristic than for Ewing, but can not serve as its pathognomonic symptom. In 70% of cases, the zone adjacent to the knee joint is affected - the distal metadiaphysis of the femur and the proximal metastiaphysis of the tibia. In 20% of cases, the proximal metadialysis of the humerus suffers. The defeat of the bones of the spine, skull and pelvis is extremely rare and is usually associated with a fatal prognosis due to the impossibility of a radical operation.
How is osteogenic sarcoma manifested?
Osteogenic bone sarcoma, which usually occurs in the central parts, destroys the cortical layer during growth and involves the soft tissues of the limb in the tumor. For this reason, by the time of diagnosis, it usually acquires a two-component structure, i.e. Consists of bone and extra-bone (soft-tissue) components. On the longitudinal section of the bone resected together with the tumor, a wide invasion of the medullary canal is detected.
According to the morphology of tumor growth, the following types of ossiogenic 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 is distinguished with a cartilaginous (chondrogenic) component. Its frequency is 10-20%. The prognosis with this variant of the tumor is worse than with osteogenic sarcoma with the absence of a cartilage component.
On the location relative to the medullary canal radiologically separated the classical, periosteal and parostal forms of the tumor.
- Osteogenic sarcoma of classical form constitutes the vast majority of cases of this disease. It is noted typical clinical and radiographic signs of the entire bone across the bone with the exit of the process to the periosteum and further into the surrounding tissues with the formation of the soft tissue component and the filling of the tumor masses of the medullary canal.
- Osteogenic sarcoma of periosteal form is characterized by damage to the cortex without spreading the tumor to the medullary canal. Perhaps, this form should be considered as an option or stage of development of a classic tumor until it spreads to the medullary canal.
- The parosteal osteogenic sarcoma originates from the cortical layer and extends along the periphery of the bone, not penetrating into the depth of the cortical layer and into the medullary canal. The prognosis for this form, which is 4% of all diseases and occurs mainly at an older age, is better than in the classical form.
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How is osteogenic sarcoma treated?
The strategy of treatment since the description of this tumor Ewing in 1920 to the 70's. XX century. Was limited to organ-carrying operations - amputations and exarticulations. According to the literature, the 5-year survival rate with such tactics did not exceed 20%, and the frequency of local relapses reached 60%. The death of patients occurred, as a rule, within 2 years due to metastatic lesions of the lungs. From modern positions, taking into account the ability of osteogenic sarcoma to early hematogenous metastasis. The possibility of a favorable outcome without the use of chemotherapy is unlikely.
In the early 70's. XX century. There were reports of the first cases of effective chemotherapeutic treatment of this tumor. Since then, the position was firmly established that, like in other malignant tumors in children, with osteogenic sarcoma, only treatment involving polychemotherapy could be successful. Currently, the treatment has proven the effectiveness of anthracyclic antibiotics (doxorubicin), alkylating agents (cyclophosphamide, ifosfamide). Derivatives of platinum (cisplatin, carboplatin), topoisomerase (etoposide) inhibitors and high doses of methotrexate. Chemotherapy is performed in nasoadjuvant (before surgery) and adjuvant (after surgery) regimens. Regional (intraarterial) chemotherapy is not accompanied by a higher efficacy compared with systemic (intravenous). With this in mind, an intravenous infusion of cytostatics should be preferred. Osteogenic sarcoma refers to radioresistant tumors, radiation therapy is not applied to it.
The question of the sequence of the surgical and chemotherapeutic stages of pulmonary metastasis treatment of osteogenic sarcoma is decided depending on the severity and dynamics of the metastatic process. In those cases when up to four metastases are detected in one lung, treatment should be started with the removal of metastases followed by chemotherapy. When more than four metastases are found in an easy or bilateral metastatic lesion, chemotherapy is initially performed followed by a possible radical operation. In the initially unresectable nature of metastatic lung disease, the presence of tumor pleurisy and the persistence of non-resectability of metastases after chemotherapeutic treatment, the attempt to surgically treat metastases is inappropriate.
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What prognosis does osteogenic sarcoma have?
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 the female. Osteogenic sarcoma has a worse prognosis, the bigger the bone in which it originated, which is associated with the possible longer latent course of the tumor process and the achievement of a larger mass of tumor cells before the stage of diagnosis and the initiation of special treatment.
At present, 70% of the overall 5-year survival rate in the case of osseogenic sarcoma has been achieved, provided that the program treatment is carried out. The survival indices, depending on the organ-carrying or organ-preserving operation, do not differ. With metastatic lung disease, the overall 5-year survival rate is 30-35%. Early appearance (within 1 year from the end of treatment) of metastases to the lungs to a greater extent reduces the likelihood of recovery, compared with the diagnosis of metastases at a later date. The prognosis is fatal in case of unresectable pulmonary metastases, metastatic damage to bones and lymph nodes, non-responsiveness of the primary tumor. The prognosis is worse the younger the patient. Girls recover more often than boys.