Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges to the investigator, clinician, and patient. One consequence of their rarity among sarcomas, as well as their biologic and clinical heterogeneity, is that management guidelines are inadequate to inform the range of individual patient-treatment decisions from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence, palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among the most curable, with more than two-thirds of patients with localized disease likely to achieve long-term survival. Neoadjuvant chemotherapy comprising cisplatin, doxorubicin, and methotrexate with intercalated surgery is the standard of care for resectable OS in those younger than 40 years. Outcomes for OS presenting with unresectable metastases or recurrent disease, or in those older than 40 years are generally poor. Overall results have improved little for all patients with OS, and new treatments are needed. Surgical resection remains the cornerstone of management for chondrosarcoma and chordoma. However, the application of new biologic insights to therapeutic development indicates that improved treatments may soon be routine for patients with chondrosarcoma and chordoma for whom surgery alone is inadequate. For all these uncommon diseases, patients should be offered specialist expert care delivered by experienced multidisciplinary teams in high-volume centers.

中文翻译：

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骨肉瘤、软骨肉瘤和脊索瘤

骨肉瘤 (OS)、软骨肉瘤和脊索瘤的特点是对研究者、临床医生和患者的多重挑战。它们在肉瘤中很少见，以及它们的生物学和临床异质性的一个后果是，管理指南不足以告知个体患者治疗决策的范围，从诊断、手术方法、化疗、放疗、复发治疗、姑息治疗和生存质量。在高级别肉瘤中，OS 是最可治愈的，超过三分之二的局部疾病患者可能实现长期生存。包括顺铂、多柔比星和甲氨蝶呤在内的新辅助化疗联合介入手术是 40 岁以下可切除 OS 的标准治疗。出现不可切除转移或复发性疾病或 40 岁以上的 OS 的结果通常很差。所有 OS 患者的总体结果几乎没有改善，需要新的治疗方法。手术切除仍然是软骨肉瘤和脊索瘤治疗的基石。然而，新的生物学见解在治疗开发中的应用表明，改进的治疗方法可能很快成为仅靠手术不足以治疗软骨肉瘤和脊索瘤的患者的常规治疗方法。对于所有这些不常见的疾病，应该为患者提供由经验丰富的多学科团队在高容量中心提供的专科专家护理。并且需要新的治疗方法。手术切除仍然是软骨肉瘤和脊索瘤治疗的基石。然而，新的生物学见解在治疗开发中的应用表明，改进的治疗方法可能很快成为仅靠手术不足以治疗软骨肉瘤和脊索瘤的患者的常规治疗方法。对于所有这些不常见的疾病，应该为患者提供由经验丰富的多学科团队在高容量中心提供的专科专家护理。并且需要新的治疗方法。手术切除仍然是软骨肉瘤和脊索瘤治疗的基石。然而，新的生物学见解在治疗开发中的应用表明，改进的治疗方法可能很快成为仅靠手术不足以治疗的软骨肉瘤和脊索瘤患者的常规治疗方法。对于所有这些不常见的疾病，应该为患者提供由经验丰富的多学科团队在高容量中心提供的专科专家护理。