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Osteogenic sarcoma
Last reviewed: 04.07.2025
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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 that form malignant osteoid.
ICD-10 code
- C40. Malignant neoplasm of bone and articular cartilage of extremities.
- C41. Malignant neoplasm of bone and articular cartilage of other and unspecified sites.
[ What causes osteosarcoma?
Most cases of the disease are spontaneous, but the importance of radiation exposure and previous chemotherapy in the subsequent development of osgeogenic sarcoma has been proven, as well as a link with the presence of the reticoblastoma gene RB-1. Increased incidence is recorded in people with Paget's disease (deforming osteosis) and Ollier's disease (dyschondroplasia).
A typical source of tumor growth is the metadiaphyseal growth zone. Metadiaphysis damage is more typical for osteogenic sarcoma than for Ewing, but cannot 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 metadiaphysis of the tibia. In 20% of cases, the proximal metadiaphysis of the humerus is affected. Damage to the bones of the spine, skull and pelvis occurs extremely rarely and is usually associated with a fatal prognosis due to the impossibility of radical surgery.
How does osteosarcoma manifest itself?
Osteogenic bone sarcoma, which usually occurs in the central sections, destroys the cortex 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 extraosseous (soft tissue) components. A longitudinal section of the bone resected together with the tumor reveals extensive invasion of the medullary canal.
According to the morphology of tumor growth, the following types of osteogenic sarcoma are distinguished: osteoplastic (32%), osteolytic (22%) and mixed (46%). These morphological types correspond to variants of the radiographic picture of the tumor. The survival of patients does not depend on the radiomorphological variant. The disease with a cartilaginous (chondrocnemius) component is distinguished separately. Its frequency is 10-20%. The prognosis for this tumor variant is worse than for osteogenic sarcoma with the absence of a cartilaginous component.
Based on the location relative to the bone marrow canal, radiologically, the tumor is divided into classical, periosteal and parosteal forms.
- Osteogenic sarcoma of the classical form constitutes the overwhelming majority of cases of this disease. It is characterized by typical clinical and radiological signs of damage to the entire cross-section of the bone with the process extending to the periosteum and further into the surrounding tissues with the formation of a soft tissue component and filling the bone marrow canal with tumor masses.
- Osteogenic sarcoma of the periosteal form is characterized by damage to the cortical layer without tumor spread to the medullary canal. Perhaps this form should be considered a variant or stage of development of a classic tumor before its spread to the medullary canal.
- Parosteal osteogenic sarcoma originates from the cortex and spreads along the periphery of the bone without penetrating deep into the cortex or into the medullary canal. The prognosis for this form, which accounts for 4% of all cases and occurs primarily in older age, is better than for the classic form.
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How is osteosarcoma treated?
The treatment strategy from the time of the description of this tumor by Ewing in 1920 until the 1970s was limited to organ-removing 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 lung damage. From a modern standpoint, taking into account the ability of osteosarcoma to early hematogenous metastasis, the possibility of a favorable outcome without the use of chemotherapy is unlikely.
In the early 1970s, the first cases of effective chemotherapeutic treatment of this tumor were reported. Since then, it has become firmly established that, as with other malignant tumors in children, only treatment that includes polychemotherapy can be successful with osteosarcoma. Currently, the effectiveness of anthracyclic antibiotics (doxorubicin), alkylating agents (cyclophosphamide, ifosfamide), platinum derivatives (cisplatin, carboplatin), topoisomerase inhibitors (etoposide) and high doses of methotrexate has been proven in treatment. Chemotherapy is administered in non-adjuvant (before surgery) and adjuvant (after surgery) modes. Regional (intra-arterial) chemotherapy is not more effective than systemic (intravenous). Taking this into account, intravenous infusion of cytostatics should be preferred. Osteogenic sarcoma is a radioresistant tumor and radiation therapy is not used for it.
The question of the order of surgical and chemotherapeutic stages of treatment of pulmonary metastases of osteosarcoma is decided depending on the severity and dynamics of the metastatic process. In cases where up to four metastases are detected in one lung, it is advisable to begin treatment with removal of metastases followed by chemotherapy. If more than four metastases are detected in the lung or bilateral metastatic lesions, chemotherapy is initially carried out followed by possible radical surgery. In the case of initially unresectable nature of metastatic lung lesions, presence of tumor pleurisy and persistence of unresectable metastases after chemotherapeutic treatment, an attempt at surgical treatment of metastases is inappropriate.
Drugs
What is the prognosis for osteosarcoma?
The prognosis is better in the age group from 15 to 21 years compared to younger patients, in all age groups the prognosis is better in females. Osteogenic sarcoma has a worse prognosis, the larger the bone in which it arose, which is associated with a 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 beginning of special treatment.
Currently, 70% overall 5-year survival rate for osteogenic sarcoma has been achieved, provided that programmatic treatment is carried out. Survival rates do not differ depending on organ-removing or organ-preserving surgery. In case of metastatic lung lesions, the overall 5-year survival rate is 30-35%. Early appearance (within 1 year from the end of treatment) of metastases to the lungs significantly 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 bone and lymph node lesions, and non-resectability of the primary tumor. The prognosis is worse, the younger the patient. Girls recover more often than boys.