骨源性肉瘤:过去、现在和未来。

M M Copeland, W W Sutow
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摘要

成骨肉瘤(osteogenic sarcoma, osteosarcoma)一词适用于恶性骨形成肿瘤,可通过所产生的基质来识别,尽管组织学模式可能有很大不同。这种细胞型(溶骨型)肿瘤预后较差。其他影响预后的因素有:1)原发肿瘤的部位;2)症状持续时间;3)病变程度及肿瘤大小。肿瘤起源于骨骺线干骺端。肿瘤的组织发生解释了这种分布。诊断活检后,截肢仍然是治疗的选择。在选定的病例中,可能会出现保留肢体的根治性整体切除。在某些类型的骨肉瘤(尤文氏肉瘤和原发性骨网状细胞肉瘤)中,放疗作为辅助治疗和主要决定性治疗的作用较小,但也很重要。直到最近,化疗药物仅用于晚期缓解。然而,治疗的进步是由于创新的术后辅助化疗在儿童中的应用。讨论了成人和儿童截肢后的各种化疗方案。在大多数仅截肢后的此类病例中,5年生存率不超过15-20%,在致命病例中,疾病在18个月内复发。目前的研究反映了更有效的辅助化疗方案,并改善了转移性骨肉瘤的姑息效果。尽管生存期大大延长,但许多患者在经过长时间的控制后出现疾病复发,这表明5年的生存期可能并不意味着完全治愈。在M.D. Anderson医院,所有非转移性疾病患者的3年预计总生存率为79%。这些结果来自于康普瑞i和康普瑞ii化疗方案的使用。更强化的治疗可能产生更高的存活率。众所周知,患者的免疫状态与预后密切相关。虽然大多数免疫治疗的研究都是初步的,但重点放在改善免疫缺陷患者的免疫系统上。
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Osteogenic sarcoma: the past, present, and future.

The term osteogenic sarcoma (osteosarcoma) is applied to malignant bone-forming tumors, identifiable by the matrix produced, though the histologic pattern may differ greatly. Ths more cellular (osteolytic) forms of the tumor have the poorer prognosis. Other prognostic factors are 1) site of the primary tumor; 2) duration of symptoms; and 3) extent of disease and tumor size. The site of tumor origin is the metaphyseal side of the epiphyseal line. The histogenesis of the tumors accounts for this distribution. Following a diagnostic biopsy, amputation of the extremities remains the treatment of choice. In selected cases, a limb-saving radical en bloc resection may surface. Radiotherapy plays a lesser but important role as adjunctive treatment, and as primary definitive treatment in certain types of bone sarcoma (Ewing sarcoma and primary reticulum cell sarcoma of bone). Until recently, chemotherapeutic agents have been used for late palliation only. Advances in treatment, however, have resulted from the application of innovative postsurgical adjuvant chemotherapy in children. The various chemotherapeutic regimens following amputation in adults and in children are discussed. In most such series of cases following amputation alone, five-year survivals have not exceeded 15-20%, with recurrent disease appearing within 18 months in fatal cases. Current studies reflect more effective regimens of adjuvant chemotherapy, with improved palliative results in metastatic osteogenic sarcoma. Although survival is much prolonged, however, many patients show a recurrence of the disease after long intervals of control, suggesting that five-year survival may not indicate a complete cure. At M.D. Anderson Hospital, th projected overall survival rate at three years is 79% of all patients with nonmetastatic disease. These results have accrued from the use of Compadri-I and Compadri-II regimens of chemotherapy. More intensive therapy may yield higher survival rates. It is known that the immunologic status of a patient definitely relates to prognosis. Although most of the investigations with immunotherapy are preliminary, emphasis is placed on improving the immune system in immunodeficient patients.

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