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Key Features

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  • Accounts for 60% of cases and occurs mostly in adolescents and young adults

  • The metaphyses of long tubular bones are primarily affected

  • The distal femur accounts for more than 40% of cases, with the proximal tibia, proximal humerus, and mid and proximal femur following in frequency

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Clinical Findings

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  • Pain over the involved area is the usual presenting symptom with or without an associated soft tissue mass

  • Patients generally have symptoms for several months prior to diagnosis

  • Cardinal signs of bone tumor

    • Pain at the site of involvement, often following slight trauma

    • Mass formation

    • Fracture through an area of cortical bone destruction

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Diagnosis

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  • Laboratory findings may reveal elevated serum alkaline phosphatase or lactate dehydrogenase levels

  • Radiographic findings show

    • Permeative destruction of the normal bony trabecular pattern with indistinct margins

    • Periosteal new bone formation and lifting of the bony cortex may create a Codman triangle

    • Soft tissue mass plus calcifications in a radial or sunburst pattern

  • MRI is more sensitive than CT scanning in defining the extent of the primary tumor

  • CT scan of the chest and bone scan are essential for detecting metastatic disease

  • Positron emission tomography (PET)-CT may be a consideration in monitoring response to therapy

  • Bone marrow aspirates and biopsies are not indicated

  • Tissue sample is needed to confirm the diagnosis

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Treatment

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  • Chemotherapy is often administered prior to definitive surgery (neoadjuvant chemotherapy)

    • May be administered intra-arterially or intravenously, although the benefits of intra-arterial chemotherapy are disputed

    • Effective agents include doxorubicin, cisplatin, high-dose methotrexate, ifosfamide, and etoposide

  • Definitive cure requires en bloc surgical resection of the tumor with a margin of uninvolved tissue

  • Amputation, limb salvage, and rotationplasty (Van Ness rotation) are equally effective in achieving local control of osteosarcoma

  • Contraindications to limb-sparing surgery include

    • Major involvement of the neurovascular bundle by tumor

    • Immature skeletal age, particularly for lower extremity tumors

    • Infection in the region of the tumor

    • Inappropriate biopsy site

    • Extensive muscle involvement that would result in a poor functional outcome

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