INTERGROUP RHABDOMYOSARCOMA STUDY

横纹肌肉瘤组间研究

• 批准号: 6310917
• 负责人: WILLIAM M CRIST
• 金额: $ 62.04万
• 依托单位: NATIONAL CHILDHOOD CANCER FOUNDATION
• 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 2002-12-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6310917
• 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 美国国税局自1972年成立以来， 是一项由儿童基金会开展的多学科合作研究， 癌症组（CCG）和儿科肿瘤组（POG）。资格 CCG和POG机构的患者在IRS上登记 协议. 有四个主要的方案研究：IRS-I（1972- IRS-II（1978-84）、IRS-III（1984 - 1991）和IRS-IV（1991-__），加上 干预试点研究协议。第四个方案，IRS-IV，开始了 1991年10月。 在686至1062名合格患者中， 入组每项完整研究（IRS-I、-II、-III）。每个方案开始时 与IRS-II，作为前面的研究的产物开发。的 IRS-I、IRS-II、IRS-III的结果揭示了各种外科手术， 放射治疗和化学治疗方面的关系 疾病阶段、原发肿瘤部位、肿瘤组织学和 疾病传播。 患者生存期和完全缓解（CR）持续时间 从IRS-I到-II再到- III，对确定的风险组进行增量强化治疗。 在3年时，IRS-III组73%的总生存率上级优于 IRS-II，67%和IRS-I，60% p<.001。 同样的关系也适用于 CR持续时间（76%对69%对64%，p<.001）。 IRS-IV将招募患者 在5年的时间里。 一种新的IRS衍生TNM治疗前分期 分类决定治疗随机化。 主要 化疗问题是（l）比较疗效 环磷酰胺vs异环磷酰胺vs异环磷酰胺+VP-16，和（2）秩 诱导药物双联的顺序（长春新碱/美法仑vs 异环磷酰胺/依托泊苷vs异环磷酰胺阿霉素）及其临床 对VAC治疗的交叉抗性。 一个主要的放射治疗问题也 讨论：超分割与常规剂量。 患者随访- 数据分析将继续进行，直到所有患者都 从治疗开始至少随访5年。 晚 治疗效果正在监测和确定在选定的 患者亚组。 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 来检验治疗假说、分子遗传学和其他肿瘤 生物学研究，免疫组织化学，和迟发效应。试点研究 已经开始了对IRS-V的新疗法。 IRS-V应该 开始于1997年。