BIOLOGY AND TREATMENT OF PEDIATRIC SOFT TISSUE AND EWING'S SARCOMA

包括尤因肉瘤在内的儿童肉瘤的研究， 横纹肌肉瘤、未分化肉瘤以及其他肉瘤， 目前正在两个领域开展工作：生物学研究和治疗试验。 生物学研究涉及：1)体外组织培养评价和 这些患者肿瘤细胞系的特征 肉瘤细胞系体外分化的研究 患有这些肉瘤的患者；3)发展抗肉瘤单抗 儿童肉瘤；4)儿童肉瘤的细胞遗传学定义 肉瘤；5)来源细胞系的体外辐射和化疗敏感性 来自这些肉瘤患者的肿瘤；6)分子生物学 对这些肿瘤的研究。尤文氏病的细胞遗传学特征 肉瘤，周围神经上皮瘤已证实易位 T(11；22)在所有研究这些疾病的细胞系中。 治疗研究的目的是：1)改善患者的治疗 高危儿童肉瘤a)通过改进最初的诱导 使用密集诱导法的比率和b)通过利用 强化巩固，包括大剂量化疗，全身 放射治疗和自体骨髓回输；2)改善 中等风险儿童肉瘤患者的治疗；3)改进 在检测、评估和治疗肺转移瘤中的作用 儿童肉瘤患者；4)仔细评估短小和 化疗和全身放射治疗对心脏的远期影响 和肺功能，以及其他主要器官系统；5)评估 自体骨髓移植的疗效和毒性 一种新型化疗药物治疗儿童肉瘤的临床研究 放射治疗方案。 自1983年初83-C-73协议开始以来，已有70多名患者 已输入。对于新诊断的患者，最初的完全缓解 缓解率超过93%，仍有70%以上处于缓解期。 这些数字代表着比以前在这个国家使用的方案有所改善 机构。

The study of pediatric sarcomas including Ewing's sarcoma, rhabdomyosarcoma, and undifferentiated sarcomas, as well as other sarcomas, is being undertaken in two areas: biological studies and therapeutic trials. The biological studies address: 1) in vitro tissue culture evaluation and characterization of the cell lines from tumors of patients with these sarcomas; 1) in vitro differentiation of cell lines derived from tumors of patients with these sarcomas; 3) development of monoclonal antibodies to pediatric sarcomas; 4) definition of the cytogenetics of pediatric sarcomas; 5) in vitro radiation and chemosensitivity of cell lines derived from tumors of patients with these sarcomas; and 6) molecular biologic studies of these tumors. The cytogenetic characterization of Ewing's sarcoma, peripheral neuroepithelioma has confirmed the translocation t(11;22) in all cell lines studied with these disorders. The therapeutic studies address: 1) improvement in therapy for patients with high risk pediatric sarcomas a) by improving the initial induction rate using an intensive induction and b) by improving survival by utilizing intensive consolidation including high dose chemotherapy, total body radiotherapy, and autologous bone marrow reinfusion; 2) improvemnt in therapy for patients with moderate risk pediatric sarcomas; 3) improvement in the detection, evaluation, and treatment of pulmonary metastasis in patients with pediatric sartcomas; 4) careful evaluation of the short and long term effects of chemotherapy and total body radiotherapy on cardiac and pulmonary function, as well as other major organ systems; 5) evaluation of the efficacy and toxicity of autologous bone marrow transplantation in the treatment of pediatric sarcomas using a new chemotherapeutic and radiotherapeutic regimen. Since the 83-C-73 protocodl was begun in early 1983 over 70 patients have been entered. For newly diagnosed patients the initial complete remission rate has been greater than 93% and greater than 70% remain in remission. These figures represent improvement over previous regimens utilized at this institution.