Localized Osteosarcoma / Malignant Fibrous Histiocytoma of Bone
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ Pediatric and Adult Treatment Editorial Boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Patients with localized osteosarcoma undergoing surgery and chemotherapy have a 5-year overall survival (OS) of 65% to 70%.[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] 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]
General Information About Langerhans Cell Histiocytosis (LCH)
The National Cancer Institute (NCI) provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. The histiocytic diseases in children and adults include three major classes of disorders of which only one, Langerhans cell histiocytosis (LCH), a dendritic cell disorder, will be discussed. Others in this category are Erdheim-Chester disease (primarily found...
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While a needle biopsy can often make the diagnosis of osteosarcoma, participation in clinical trials may require collection of material for biologic studies that can only be obtained via an open biopsy or multiple needle biopsies. If limb sparing (removal of the malignant bone tumor without amputation and replacement of bones or joints with allografts or prosthetic devices) is contemplated, the biopsy should be performed by the surgeon who will do the definitive operation, since incision placement is crucial.
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 should be planned only when the preoperative staging indicates that it would be possible to achieve wide surgical margins. 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 in resumption of chemotherapy after definitive surgery is associated with increased risk of tumor recurrence and death.[9][Level of evidence: 1iiA] For some patients, amputation remains the optimal choice for management of the primary tumor. A pathologic fracture noted at diagnosis or during preoperative chemotherapy does not preclude limb-salvage surgery if wide surgical margins can be achieved.[10] In two series, patients presenting with a pathologic fracture at diagnosis had similar outcomes to those without pathologic fractures at diagnosis, while in a third series, pathologic fracture at diagnosis was associated with a worse overall outcome.[11,12]; [13][Level of evidence: 3iiiA] If the pathologic examination of the surgical specimen shows inadequate margins, an immediate amputation should be considered, especially if the histologic necrosis following preoperative chemotherapy was poor.[14] In one study, patients undergoing limb-salvage procedures who had poor histologic response and close surgical margins had a high rate of local recurrence.[15]
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