Hays D M
Department of Surgery, Childrens Hospital Los Angeles, CA 90027.
Semin Surg Oncol. 1993 Nov-Dec;9(6):520-3. doi: 10.1002/ssu.2980090610.
Rhabdomyosarcomas (RMS) occurring in pelvic sites are almost entirely of the embryonal histologic subtype and are relatively responsive to chemotherapeutic agents. When treated by radical surgery, pelvic irradiation, and intensive chemotherapy for 2 years, survival rates of higher than 85% have been achieved. However, the effects of such therapeutic approaches have resulted in major changes in the quality of life of survivors, because of the loss of pelvic organs, primarily the bladder. Current efforts are directed towards achieving long range survival with procedures that are less extensive than pelvic exenteration or total cystectomy and by regimens that eliminate or reduce the intensity of radiotherapy. Until the past 5 years, these efforts have been relatively unsuccessful in both North America and Europe. However, the most recent studies of the IRS and European cooperative groups suggest that a majority of these patients can survive with intact pelvic organs when treated with currently available chemotherapeutic agents in conjunction with radiotherapy and limited surgery.
发生于盆腔部位的横纹肌肉瘤（RMS）几乎全部为胚胎组织学亚型，并且对化疗药物相对敏感。通过根治性手术、盆腔放疗和强化化疗治疗2年，生存率已超过85%。然而，由于盆腔器官（主要是膀胱）的缺失，这些治疗方法的效果导致了幸存者生活质量的重大改变。目前的努力方向是通过比盆腔脏器清除术或全膀胱切除术范围更小的手术以及消除或降低放疗强度的方案来实现长期生存。直到过去5年，在北美和欧洲，这些努力相对都不太成功。然而，美国横纹肌肉瘤研究（IRS）和欧洲合作组的最新研究表明，当使用目前可用的化疗药物联合放疗和有限的手术进行治疗时，这些患者中的大多数可以在盆腔器官完整的情况下存活。
