OSTEOSARCOMA LÀ GÌ

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Cystic + sclerotic/ blastic lesions in the epiphysial of the right femur, both in the medullary cânl and the cortex Codman’s triangle sign nearby soft tissue thickening  Osteosarcomas are malignant bone forming tumours và the second most common primary bone tumour after multiple myeloma . Osteosarcoma accounts for approximately 20% of primary malignancies of bone. The incidence is approximately 1 lớn 3 per 1 million per year. Primary high-grade osteosarcoma occurs most commonly in the second decade of life. Parosteal osteosarcoma has a peak incidence in the third và fourth decades, and secondary osteosarcomas are more comtháng in older individuals. The incidence is slightly higher in males (with the exception that parosteal osteosarcoma is more common in females). No bone is immune lớn develop osteosarcoma, commonly develops at sites of rapid bone growth where mitotic activity is high. The most common sites involved are distal femur, proximal tibia, proximal humerus. More than 50% lesions occurs around knee Secondary osteosarcomas are equally distributed in all bones Metaphysis 91% diaphysis Epiphyseal involvement is extremely rare No specific etiongắn gọn xúc tích cause developing osteosarcoma is not established till now. But some genetic abnormalities are associated with increased incidence of these tumors Hereditary Retinoblastoma (1000 times), Li-fraumeni syndrome (p53 mutation), Rothmund-Thomson syndrome. 3q, 13q, 17p & 18q are most commonly Involved. Secondary osteosarcomas are commonly associated with Pagets disease Irradiation Fibrous dysplasia The incidence of osteosarcoma in Paget disease is approximately 1% and may be higher (5% khổng lồ 10%) and the pelvis is the most comtháng location. Radiation-induced osteosarcoma occurs in approximately 1% of patients who have sầu been treated with greater than 2500 cGy & can occur in unusual locations, such as the skull, spine, clavicle, ribs, scapula, và pelvis. osteosarcoma is the most common radiation-induced sarcoma. Other conditions that have been reported lớn be associated with secondary osteosarcomas include bone infarcts, osteochondromas, chronic osteomyelitis, dedifferentiated chondrosarcomas, melorheostosis, và osteogenesis imperfecta. Phân loại Site of origin in bone Degree of differentiation: High or Low grade Multicentrithành phố Histological variant Primary or Secondary Classic osteosarcoma is a primary Intramedullary solitary high grade osteoblastic variant arising from metaphysis of long bones The tumor ordinarily begins developing in the medullary cavity of a long bone near the metaphysis, Then, they penetrated và extended through the cortex to raising the periosteum In more advanced cases the periosteal barrier may be broken, và a soft tissue tumor mass may be seen invading the adjacent muscle tissue The articular hyaline cartilage serves as barrier to lớn prevent the extension of the neoplasm into the joint The tumor may enter the joint, however, by extending along ligamentous and capsular structures Skip metastases may occur. Progressive sầu pain is the first & most comtháng presenting symptom. Pain initially may improve with conservative sầu measures & activity modifications. Night pain may be an important clue lớn the true diagnosis; however, only about 25% of patients experience this phenomenon. Some times patient presents with a lump Pathological fracture is very unusual presentation Plain radiograph is sufficient to diagnose osteosarcoma most of the times. But CT, MRI, Bone scan, CT Chest are useful in staging of the tumor và planning of treatment. Apart from routine blood investigations Serum alkaline phosphatase, LDH are valuable và indicates poor prognosis. Osteosarcoma predominantly osteoblastic lesion at metaphyseal region of long bone. Although the lesion can be either predominantly blastic or predominantly lytic, more commonly areas of bone production & bone destruction are present. The lesion usually is quite permeative, and the borders are ill defined. Tumor pierces the cortex, & extover into adjucent soft-tissue. Periosteal reaction may take the form of “Codman's triangle,” or it may have sầu a “sunburst” or “hair on end” appearance Pathological fracture may be seen. Hair on over, Sunray, Codman’s triangle Codman, Sunray metaphysial Blastic lesion metaphysial & diaphysial Pathological fracture MRI demonstrates the degree of soft tissue extension và the relationship of the extracompartmental tumor khổng lồ fascial planes và neurovascular structures. The best feature of MRI is its ability lớn precisely evaluate the extent of tumor in the medullary cavity. Occult skip metastases of 2 milimet or more in long bones are well seen on MRI. MRI is also useful in evaluating the adjacent joint for tumor spread. Approximately 10% lớn 20% of patients with osteosarcoma present with radiographically detectable metastases at diagnosis. Most of these are in the lungs. Conventional radiographs of the chest show metastatic nodules 10 milimet or greater in diameter. Pulmonary metastases 3 to 7 milimet or greater in diameter are identified with CT. Bone scan with technetium 99m shows a marked increase in the uptake of the radionuclide in the primary tumor. The increased uptake is due lớn active formation of new tumor & host bone as well as the vascularity of the lesion. Radionuclide bone scintigraphy is used lớn look for bony metastases in the involved bone (skip metastases)& at other skeletal sites. Mineralized metastases are more likely khổng lồ be detected by bone scans than are nonmineralized ones at extrapulmonary sites. The factors which influence prognosis other than staging system are Location of the tumor Proximity lớn neurovascular structures Race (blacks > whites ) Secondary > Primary Elevated LDH & Alkaline phosphatase Presence of skip lesions Treatment options for osteosarcoma are Surgery Chemotherapy Radiotherapy in some cases Historical data suggests that survival rate with ablative sầu surgery alone is Chemotherapy has excellent role in terminating these lesions. The survival rate with chemotherapy alone is very disappointing Chemotherapy applied in two modalities Neoadjuvant chemotherapy Adjuvant chemotherapy Preoperative sầu chemotherapy causes regression of the primary tumor, making a limb salvage operation easier. Neoadjuvant chemotherapy usually can be started immediately lớn prevent tumor progression, which may occur during any delay before surgery This allows time to plan the operation properly, including the possible manufacturing of a custom implant. The aim of tumor surgery is to lớn achieve wide margins whenever possible. Main hazard of narrow margins of excision is local recurrence, which requires further surgery even amputation. It is an intermediate-grade malignancy that arises on the surface of the bone The most common locations are the diaphyses of the femur & tibia, và comtháng in a age group of around 30-5oyrs. It presents with swelling và local pain. Histological examination of periosteal osteosarcoma shows strands of osteoid-producing spindle cells radiating between lobules of cartilage. Treatment is same as classic osteosarcoma. It is a rare, low-grade malignancy, arising on the surface of the bone and invades the medullary cavity only at a late stage. It comtháng in young adults X-ray shows bony mass on the surface of the bone, usually thin gap remain between cortex & tumor. cortex is not eroded Differential diagnosis is Myositis ossificans Osteochondroma CT và MRI helpful in differentiating parosteal osteosarcoma from these lesions In myositis ossificans mature bone is at the periphery of the lesion, whereas in parosteal osteosarcoma centre of the lesion is more heavily matured. Parosteal osteosarcoma can be easily differentiated from an osteochondroma by showing medullary cavity containing marrow in continuity with the medullary canal of the involved bone. Telangiectatic osteosarcoma is a highly malignant variant of osteosarcoma . It is twice as comtháng in males than in females & comtháng in 20-30 age group. It is characterized by a high degree of vascularity & large cystic spaces filled with blood. It is an osteolytic destructive sầu lesion with an almost complete absence of sclerotic changes; a soft-tissue mass may also be present Differential diagnosis is cystic bone lesions like simple bone cyst, aneurysmal bone cyst Treatment is same as classic osteosarcoma It is rare variant of osteosarcoma which is a high-grade lesion that consists of small xanh cells that may resemble Ewing sarcoma or lymphoma. Distal femur, proximal humerus are sites of prediliction. Cytogenetic and immunohistochemistry studies sometimes are needed lớn differentiate these lesions Secondary osteosarcomas are commonly associated with Pagets disease Irradiation Fibrous dysplasia Comtháng in elderly people after age 60