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

包括尤文氏肉瘤在内的小儿肉瘤的研究， 横纹肌肉瘤和未分化肉瘤，以及其他肉瘤， 目前正在两个领域进行：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 来自患有这些疾病的患者的肿瘤的细胞系的表征 肉瘤; 1）来源于肿瘤的细胞系的体外分化， 这些肉瘤患者; 3）单克隆抗体的发展， 儿童肉瘤; 4）儿童肉瘤细胞遗传学的定义 肉瘤; 5）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;以及6）分子生物学 这些肿瘤的研究。 尤文氏瘤的细胞遗传学特征 肉瘤，周围神经上皮瘤已经证实了易位 t（11; 22）。 治疗研究的目的是：1）改善患者的治疗 a）通过改善初始诱导， 和B）通过利用强化诱导提高存活率 强化巩固治疗，包括大剂量化疗，全身 放射治疗和自体骨髓回输; 2）改善 治疗中度风险的儿童肉瘤患者; 3）改善 在肺转移的检测、评估和治疗中， 儿童肉瘤患者; 4）仔细评估短， 化疗和全身放疗对心脏病的远期影响 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， 使用新的化疗药物治疗小儿肉瘤， 放疗方案 自1983年初开始实施83-C-73方案以来， 已经进入。 对于新诊断的患者，初始完全缓解 率已超过93%，超过70%的缓解。 这些数字代表了在本研究中使用的先前方案的改善。 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.