INTERGROUP RHABDOMYOSARCOMA STUDY

横纹肌肉瘤组间研究

• 批准号: 6137381
• 负责人: WILLIAM M CRIST
• 金额: $ 6.56万
• 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 2000-02-29
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6137381
• 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.

跨组横纹肌肉瘤研究(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。美国国税局自1972年以来一直存在， 是一项由Childrens进行的多学科合作研究 癌症组(CCG)和儿科肿瘤组(POG)。合资格 CCG和POG机构的患者在IRS上登记 协议。有四项主要的议定书研究：IRS-I(1972-- 78)、IRS-II(1978-84年)、IRS-III(1984-1991年)和IRS-IV(199l-__)，加上 介入性的飞行员研究方案。第四个议定书，IRS-IV，开始 1991年10月。已有686至1062名符合条件的患者 参加每项全面研究(IRS-I、-II、-III)。每个协议开始 使用IRS-II，作为前一项研究的结果而开发。这个 IRS-I，-II，-III的结果揭示了各种外科手术， 放射治疗与化疗的相互关系 与疾病分期、原发肿瘤部位、肿瘤组织学及分型有关 疾病蔓延。患者存活率和完全缓解(CR)持续时间 从IRS-I到IRS-II再到- III对确定的风险群体逐步加强治疗。 3年后，IRS-III的总体存活率为73%，优于 IRS-II，67%；IRS-I，60%。同样的关系也适用于 CR持续时间(76%vs 69%vs%，P&lt；OOL)。IRS-IV将增加患者 在5年的时间里。一种新的基于IRS的TNM治疗前分期方法 分类决定治疗的随机性。主 化疗的问题是(L)疗效的比较 环磷酰胺VS异环磷酰胺VS异环磷酰胺+VP-16，(2)排名 诱导药物对的排序(长春新碱/马法兰vs 异环磷酰胺/依托泊苷与异环磷酰胺、阿霉素的比较 对VAC治疗的交叉耐药性。放射治疗的一个主要问题也是 解决的是：超分割与常规剂量。患者随诊- UP和数据分析将继续进行，直到所有患者 从治疗开始至少随访5年。晚些 正在监测和确定选定的治疗效果 患者分组。IRS-V规划正在顺利进行，将包括 DNA倍体和细胞遗传学分期、风险导向治疗策略 检验治疗假说、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 已经开始了针对IRS-V的新疗法的研究。IRS-V应该 从1997年开始。