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    Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Localized Osteosarcoma and MFH of Bone

    Patients with localized osteosarcoma undergoing surgery and chemotherapy have a 5-year overall survival (OS) of 62% to 65%.[1] Complete surgical resection is crucial for patients with localized osteosarcoma; however, at least 80% of patients treated with surgery alone will develop metastatic disease.[2] Randomized clinical trials have established that adjuvant chemotherapy is effective in preventing relapse or recurrence in patients with localized resectable primary tumors.[2]; [3][Level of evidence: 1iiA]

    Patients with malignant fibrous histiocytoma (MFH) of bone are treated according to osteosarcoma treatment protocols, and the outcome for patients with resectable MFH is similar to the outcome for patients with osteosarcoma.[4] As with osteosarcoma, patients with a favorable necrosis (≥90% necrosis) had a longer survival than those with an inferior necrosis (<90% necrosis).[5] MFH of bone is seen more commonly in older adults. Many patients with MFH will need preoperative chemotherapy to achieve a wide local excision.[6]

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    The diagnosis of osteosarcoma can be made by needle biopsy, core needle biopsy, or open surgical biopsy. It is preferable that the biopsy be done by a surgeon skilled in the techniques of limb sparing (removal of the malignant bone tumor without amputation and replacement of bones or joints with allografts or prosthetic devices). In these cases, the original biopsy incision placement is crucial. Inappropriate alignment of the biopsy or inadvertent contamination of soft tissues can render subsequent limb-preserving reconstructive surgery impossible.

    Surgical Removal of Primary Tumor

    Surgical resection of the primary tumor with adequate margins is an essential component of the curative strategy for patients with localized osteosarcoma. The type of surgery required for complete ablation of the primary tumor depends on a number of factors that must be evaluated on a case-by-case basis.[7]

    In general, more than 80% of patients with extremity osteosarcoma can be treated by a limb-sparing procedure and do not require amputation.[8] Limb-sparing procedures are planned only when the preoperative staging indicates that it would be possible to achieve wide surgical margins. In one study, patients undergoing limb-salvage procedures who had poor histologic response and close surgical margins had a high rate of local recurrence.[9] Reconstruction after surgery can be accomplished with many options including metallic endoprosthesis, allograft, vascularized autologous bone graft, and rotationplasty. The choice of optimal surgical reconstruction involves many factors, including the site and size of the primary tumor, the ability to preserve the neurovascular supply of the distal extremity, the age of the patient and potential for additional growth, and the needs and desires of the patient and family for specific function, such as sports participation. If a complicated reconstruction delays or prohibits the resumption of systemic chemotherapy, limb preservation may endanger the chance for cure. Retrospective analyses have shown that delay (≥ 21 days) in resumption of chemotherapy after definitive surgery is associated with increased risk of tumor recurrence and death.[10][Level of evidence: 1iiA]

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