INTERGROUP RHABDOMYOSARCOMA STUDY

横纹肌肉瘤组间研究

• 批准号: 2806031
• 负责人: WILLIAM M CRIST
• 金额: $ 40.72万
• 依托单位: MAYO CLINIC ROCHESTER
• 依托单位国家: 美国
• 财政年份: 1979
• 资助国家: 美国
• 起止时间: 1979-01-01 至 1999-12-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2806031
• 关键词: child (0-11); clinical research; clinical trials; combination cancer therapy; combination chemotherapy; cyclophosphamide; doxorubicin; etoposide; gene mutation; human subject; human therapy evaluation; ifosfamide; neoplasm /cancer chemotherapy; neoplasm /cancer diagnosis; neoplasm /cancer genetics; neoplasm /cancer radiation therapy; neoplasm /cancer surgery; pediatric neoplasm /cancer; rhabdomyosarcoma; transcription factor

The Intergroup Rhabdomyosarcoma Study (IRS) is designed to answer important therapeutic, clinical, and laboratory research questions about rhabdomyosarcoma. The IRS has been in existence since 1972 and is a collaborative multidisciplinary study carried out by Childrens Cancer Group (CCG) and the Pediatric Oncology Group (POG). Eligible patients in CCG and POG institutions are registered on the IRS protocols. There have been four major protocol studies: IRS-I(1972- 78), IRS-II(1978-84), IRS-III(l984-l99l), and IRS-IV(199l-__), plus intervening pilot studyprotocols. The fourth protocol, IRS-IV, began in October 1991. Between 686 and 1062 eligible patients have been entered on each full study (IRS-I, -II, -III). Each protocol beginning with IRS-II, developed as an outgrowth of the preceding study. The results of IRS-I, -II, -III have shed light on various surgical, radiotherapeutic and chemotherapeutic aspects of treatment in relation to disease stage, primary tumor site, tumor histology and patterns of disease spread. Patient survival and complete remission (CR) duration have increased progressively and significantly from IRS-I to -II to - III with incremental intensification of therapy to defined risk groups. At 3 years, the overall survival rate of 73% in IRS-III is superior to IRS-II, 67% and IRS-I, 60% p<.OOl. The same relationship is true for CR duration (76% vs 69% vs 64%, p<.OOl). IRS-IV will accrue patients over a 5-year period. A new IRS-derived TNM pre-treatment staging classification determines treatment randomization. The main chemotherapy questions are (l) comparison of the efficacy of cyclophosphamide vs ifosfamide vs ifosfamide + VP-l6, and (2) rank ordering of induction drug doublets (vincristine/melphalan vs ifosfamide/etoposide vs ifosfamide doxorubicin) and their clinical cross-resistance to VAC therapy. A major radiotherapy question also is addressed: hyperfractionated vs conventional dose. Patient follow- up and data analysis will continue until all patients have been followed for a minimum of 5 years from the start of treatment. Late treatment effects are being monitored and identified in selected patient subgroups. IRS-V planning is well underway and will include DNA ploidy and cytogenetic staging, risk-directed treatment strategies to test therapeutic hypotheses, molecular genetics and other tumor biology studies, immunohistochemistry, and late effects. Pilot studies of new therapy for IRS-V have already been initiated. The IRS-V should begin in 1997.

组间横纹肌肉瘤研究 (IRS) 旨在回答 重要的治疗、临床和实验室研究问题 关于横纹肌肉瘤。 美国国税局 (IRS) 自 1972 年以来一直存在， 是一项由儿童基金会进行的多学科合作研究 癌症组 (CCG) 和儿童肿瘤组 (POG)。有资格的 CCG 和 POG 机构的患者已在 IRS 登记 协议。 有四项主要的协议研究： IRS-I（1972- 78)、IRS-II(1978-84)、IRS-III(l984-l99l) 和 IRS-IV(199l-__)，加上 干预试点研究方案。第四个协议 IRS-IV 开始 1991 年 10 月，有 686 至 1062 名符合条件的患者接受了 参加每项完整研究（IRS-I、-II、-III）。每个协议开始 IRS-II 是作为先前研究的产物而开发的。这 IRS-I、-II、-III 的结果为各种手术、 治疗的放射治疗和化疗方面的关系 疾病阶段、原发肿瘤部位、肿瘤组织学和模式 疾病传播。 患者生存和完全缓解 (CR) 持续时间 从 IRS-I 到 -II 到 - 逐步显着增加 III 对确定的风险群体进行渐进强化治疗。 3 年时，IRS-III 的总体生存率为 73%，优于 IRS-II，67％和IRS-I，60％p<.001。 同样的关系也适用于 CR持续时间(76% vs 69% vs 64%，p<.001)。 IRS-IV 将招募患者 5年多的时间里。 源自 IRS 的新 TNM 治疗前分期 分类决定治疗随机化。 主要 化疗问题有（l）疗效比较 环磷酰胺 vs 异环磷酰胺 vs 异环磷酰胺 + VP-16，以及 (2) 排名 诱导药物双联体的排序（长春新碱/美法仑 vs 异环磷酰胺/依托泊苷与异环磷酰胺阿霉素）及其临床 对 VAC 治疗的交叉耐药性。 也是放射治疗的一个主要问题 解决：超分割剂量与常规剂量。 病人跟随- 数据分析将继续进行，直到所有患者都被治愈 从治疗开始起随访至少 5 年。 晚的 正在选定的国家中监测和确定治疗效果 患者亚组。 IRS-V 规划正在顺利进行中，将包括 DNA 倍体和细胞遗传学分期、风险导向治疗策略 测试治疗假设、分子遗传学和其他肿瘤 生物学研究、免疫组织化学和晚期效应。试点研究 IRS-V 的新疗法已经开始。 IRS-V 应该 从1997年开始。