Crist W M, Garnsey L, Beltangady M S, Gehan E, Ruymann F, Webber B, Hays D M, Wharam M, Maurer H M
Childrens Cancer Study Group, Los Angeles, CA.
J Clin Oncol. 1990 Mar;8(3):443-52. doi: 10.1200/JCO.1990.8.3.443.
Prestudy patient characteristics and specific therapy of all eligible patients with rhabdomyosarcoma entered into Intergroup Rhabdomyosarcoma (RMS) Studies I (IRS-I) (1972 to 1978, n = 686) or II (IRS-II) (1978 to 1984, n = 1,002) were examined for their relationship to survival within each of the four clinical groups using univariate and multivariate analyses. The estimated survival at 5 years from the start of treatment was 56% in IRS-I and 62% in IRS-II (P = .006). The largest survival difference between studies was in patients with group III tumors (52% v 65%). The clinical group was the most important patient characteristic related to survival in both studies. Survival progressively decreased for patients from clinical group I (localized disease, completely resected) to group IV (metastatic disease at the onset). In clinical group I, the only patient characteristic consistently related to survival was histology. Patients with alveolar tumors had the poorest survival, while those with botryoid/embryonal lesions had the best survival. In clinical group II, no characteristic was consistently related to survival. In clinical group III, an orbital primary site was associated with a favorable survival. In clinical group IV, patients with genitourinary tumors had a significant survival advantage. Use of disease-free survival as an end point gave very similar results. This information, from the largest available data base on prognostic indicators in childhood RMS in the context of aggressive multimodal therapies, is being used to plan therapy in the forthcoming study (IRS-IV).
对纳入横纹肌肉瘤（RMS）研究I（IRS-I）（1972年至1978年，n = 686）或研究II（IRS-II）（1978年至1984年，n = 1,002）的所有符合条件的横纹肌肉瘤患者的研究前患者特征和具体治疗方法进行了研究，采用单因素和多因素分析来考察其与四个临床组中每组患者生存率的关系。从治疗开始起5年的估计生存率在IRS-I中为56%，在IRS-II中为62%（P = 0.006）。两项研究之间生存率差异最大的是III组肿瘤患者（52%对65%）。临床组是两项研究中与生存率相关的最重要患者特征。从临床组I（局限性疾病，完全切除）到组IV（发病时即有转移性疾病）患者的生存率逐渐降低。在临床组I中，唯一始终与生存率相关的患者特征是组织学类型。肺泡型肿瘤患者的生存率最差，而葡萄状/胚胎型病变患者的生存率最佳。在临床组II中，没有特征始终与生存率相关。在临床组III中，眼眶原发部位与良好的生存率相关。在临床组IV中，泌尿生殖系统肿瘤患者具有显著的生存优势。将无病生存率作为终点得出了非常相似的结果。这些来自关于儿童RMS预后指标的最大可用数据库、处于积极多模式治疗背景下的信息，正被用于规划即将开展的研究（IRS-IV）中的治疗方案。
