Wolden S L, Anderson J R, Crist W M, Breneman J C, Wharam M D, Wiener E S, Qualman S J, Donaldson S S
Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
J Clin Oncol. 1999 Nov;17(11):3468-75. doi: 10.1200/JCO.1999.17.11.3468.
To evaluate the outcome of patients with rhabdomyosarcoma (RMS) treated with complete surgical resection and multiagent chemotherapy, with or without local radiotherapy (RT).
Four hundred thirty-nine patients with completely resected (ie, group I) RMS were further treated with chemotherapy (vincristine and actinomycin D +/- cyclophosphamide, doxorubicin, and cisplatin) on Intergroup Rhabdomyosarcoma Studies (IRS) I to III between 1972 and 1991. Eighty-three patients (19%) also received local RT as a component of initial treatment.
Eighty-six patients relapsed (10-year failure-free survival [FFS] 79%, overall survival 89%). Six percent of failure sites were local, 6% were regional, and 7% were distant. Poor prognostic factors were tumor size greater than 5 cm, alveolar or undifferentiated histology, primary tumor sites other than genitourinary, and treatment on IRS-I or II. For patients with embryonal RMS who were treated with RT, there was a trend for improved FFS but no difference in overall survival. On IRS-I and II, patients with alveolar or undifferentiated sarcoma who received RT compared with those who did not receive RT had greater 10-year FFS rates (73% v 44%, respectively; P =.03) and overall survival rates (82% v 52%, respectively; (P =.02). Such patients who received RT on IRS III also benefited more than those who did not receive RT (10-year FFS, 95% v 69%; P =.01; overall survival, 95% v 86%; P =.23).
Patients with group I embryonal RMS have an excellent prognosis when treated with adjuvant multiagent chemotherapy without RT. Patients with alveolar RMS or undifferentiated sarcoma fare worse; however, FFS and overall survival are substantially improved when RT is added to multiagent chemotherapy (IRS-I and II). The best outcome occurred in IRS-III, when RT was used in conjunction with intensified chemotherapy.
评估横纹肌肉瘤（RMS）患者接受完整手术切除及多药化疗（无论是否联合局部放疗[RT]）后的治疗结果。
1972年至1991年间，439例接受了完整切除（即I组）的RMS患者在横纹肌肉瘤协作组研究（IRS）I至III中进一步接受了化疗（长春新碱和放线菌素D±环磷酰胺、阿霉素和顺铂）。83例患者（19%）还接受了局部RT作为初始治疗的一部分。
86例患者复发（10年无病生存率[FFS]为79%，总生存率为89%）。6%的复发部位为局部，6%为区域，7%为远处。预后不良因素包括肿瘤大小大于5cm、肺泡状或未分化组织学类型、泌尿生殖系统以外的原发肿瘤部位以及在IRS-I或II上的治疗。对于接受RT治疗的胚胎型RMS患者，FFS有改善趋势，但总生存率无差异。在IRS-I和II上，接受RT的肺泡状或未分化肉瘤患者与未接受RT的患者相比，10年FFS率更高（分别为73%对44%；P = 0.03），总生存率也更高（分别为82%对52%；P = 0.02）。在IRS III上接受RT的此类患者也比未接受RT的患者受益更多（10年FFS，95%对69%；P = 0.01；总生存率，95%对86%；P = 0.23）。
I组胚胎型RMS患者接受辅助多药化疗且不联合RT时预后良好。肺泡状RMS或未分化肉瘤患者预后较差；然而，当RT联合多药化疗时（IRS-I和II），FFS和总生存率显著提高。当RT与强化化疗联合使用时，IRS-III组的治疗效果最佳。
