横纹肌肉瘤协作组研究-II
The Intergroup Rhabdomyosarcoma Study-II.
作者信息
Maurer H M, Gehan E A, Beltangady M, Crist W, Dickman P S, Donaldson S S, Fryer C, Hammond D, Hays D M, Herrmann J
机构信息
Medical College of Virginia/Virginia Commonwealth University, Richmond 23298.
出版信息
Cancer. 1993 Mar 1;71(5):1904-22. doi: 10.1002/1097-0142(19930301)71:5<1904::aid-cncr2820710530>3.0.co;2-x.
BACKGROUND
Intergroup Rhabdomyosarcoma Study (IRS)-II, (1978-1984) had the general goals of improving the survival and treatment of children with rhabdomyosarcoma (RMS).
METHODS
Nine hundred ninety-nine previously untreated eligible patients entered the study after surgery and were randomized or assigned to therapy by IRS Clinical Group (I-IV), tumor site, and histologic type. Outcomes were compared between treatments and with results of IRS-I (1972-1978).
RESULTS
Patients in Group I, excluding extremity alveolar (EA) RMS, were randomized to standard vincristine (V), dactinomycin (A), and cyclophosphamide (C) or standard VA. At 5 years, disease-free survival (DFS) and survival (S) rates were similar between VAC and VA (DFS: 80%, 70%, P = 0.47; S: 85%, 84%, P = 0.73). Patients in Group II, excluding EA RMS, received radiation and were randomized to intensive VA or repetitive-pulse VAC. Outcomes were similar for rates of DFS (69%, 74%, P = 0.83) and S (88%, 79%, P = 0.17). Patients in Group III, excluding certain pelvic tumors, received radiation and were randomized to repetitive-pulse VAC or repetitive-pulse VAdrC-VAC (Adr, Adriamycin [doxorubicin]). Complete remission (CR) rates were close at 74%, 78%, respectively (P = 0.32), as were percentages in CR (73%) and S (66%) rates; the latter outcomes were significantly better than IRS-I (CR: 56%, P < 0.001; S: 50%, P < 0.001). Central nervous system prophylaxis for Group III patients with cranial parameningeal sarcoma increased S rate to 67% from 45% in IRS-I (P < 0.001). Patients in Group IV received the same regimens as Group III; the CR rate was 53%, 38% remained in CR and S rate was 27% with and 26% without Adr (P = 0.90). At 5 years, S rate for IRS-II, including EA and all pelvic tumors, was 63%: an 8% increase over IRS-I (P < 0.001). Outcomes by primary site were as good as, or better than, the IRS-I experience.
CONCLUSIONS
Combining all Groups and treatments in IRS-II, the major improvement in S rate at 5 years between studies was in nonmetastatic patients (71% for IRS-II versus 63% for IRS-I, P = 0.01).
背景
横纹肌肉瘤协作组研究（IRS）-II（1978 - 1984年）的总体目标是提高横纹肌肉瘤（RMS）患儿的生存率并改善其治疗效果。
方法
999例先前未接受治疗的符合条件的患者在手术后进入该研究，并根据IRS临床组（I - IV）、肿瘤部位和组织学类型进行随机分组或分配治疗方案。对不同治疗组的结果进行比较，并与IRS - I（1972 - 1978年）的结果进行对比。
结果
I组患者（不包括肢体肺泡型（EA）RMS）被随机分为接受标准长春新碱（V）、放线菌素D（A）和环磷酰胺（C）治疗组或标准VA治疗组。5年时，VAC组和VA组的无病生存率（DFS）和生存率（S）相似（DFS：80%，70%，P = 0.47；S：85%，84%，P = 0.73）。II组患者（不包括EA RMS）接受放疗，并被随机分为强化VA治疗组或重复脉冲VAC治疗组。DFS率（69%，74%，P = 0.83）和S率（88%，79%，P = 0.17）的结果相似。III组患者（不包括某些盆腔肿瘤）接受放疗，并被随机分为重复脉冲VAC治疗组或重复脉冲VAdrC - VAC治疗组（Adr，阿霉素[多柔比星]）。完全缓解（CR）率分别接近74%和78%（P = 0.32），CR患者百分比（73%）和S率（66%）也是如此；后两者结果明显优于IRS - I（CR：56%，P < 0.001；S：50%，P < 0.001）。III组患有颅旁脑膜肉瘤患者的中枢神经系统预防性治疗使S率从IRS - I的45%提高到67%（P < 0.001）。IV组患者接受与III组相同的治疗方案；CR率为53%，使用阿霉素和未使用阿霉素时分别有38%和27%的患者保持CR，S率分别为27%和26%（P = 0.90）。5年时，包括EA和所有盆腔肿瘤在内的IRS - II的S率为63%：比IRS - I提高了8%（P < 0.001）。各主要部位的治疗结果与IRS - I的经验相当或更好。
结论
综合IRS - II中的所有组和治疗方案，两项研究之间5年生存率的主要改善在于非转移性患者（IRS - II为71%，IRS - I为63%，P = 0.01）。
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