Atra A, Ward H C, Aitken K, Boyle M, Dicks-Mireaux C, Duffy P G, Mitchell C D, Plowman P N, Ransley P G, Pritchard J
Department of Haematology and Oncology, Hospitals for Sick Children, London, UK.
Br J Cancer. 1994 Nov;70(5):1004-8. doi: 10.1038/bjc.1994.438.
Twenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, especially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 months (range 34-103 months) overall survival was 73%, with no treatment-related death. The bladder salvage rate of 88% in survivors with bladder base/prostate primaries was much higher than that reported by the United States Intergroup Rhabdomyosarcoma Studies (IRS), though many of the preserved bladders did not function normally. We identified problems with both radiological and histological off-treatment monitoring. The overall accuracy of computerised tomographic (CT) scanning for prediction of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitored by serial cystoscopy. We conclude that a higher cure rate can be achieved by using intensive chemotherapy/radiotherapy and conservative surgery to treat children with pelvic RMS. Factors that might contribute to our favourable bladder salvage results, compared with those of the IRS, include (a) the fact that one of two specialist surgeons monitored and operated on all these patients and (b) our increasing awareness, during the study, that post-chemotherapy/radiotherapy histopathology and pelvic CT scan appearances may be misleading. Referral to paediatric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.
1983年至1988年间，共收治了26例先前未经治疗的盆腔横纹肌肉瘤（RMS）患儿，中位年龄3.4岁。其中14例为女孩。计划采用的策略是通过术前化疗、保守手术以及多数情况下的放疗来保留盆腔器官，尤其是膀胱。中位随访71个月（范围34 - 103个月），总生存率为73%，无治疗相关死亡。膀胱底部/前列腺原发性肿瘤幸存者的膀胱保留率为88%，远高于美国横纹肌肉瘤协作组研究（IRS）报告的结果，尽管许多保留的膀胱功能并不正常。我们发现了放疗后影像学和组织学监测方面的问题。计算机断层扫描（CT）预测肿瘤复发的总体准确率仅为81%，在通过系列膀胱镜检查监测的10例膀胱底部/前列腺患者中，有4例经内镜活检结果出现误导。我们得出结论，采用强化化疗/放疗和保守手术治疗盆腔RMS患儿可获得更高的治愈率。与IRS相比，可能促成我们良好膀胱保留结果的因素包括：（a）所有这些患者均由两名专科外科医生中的一名进行监测和手术；（b）在研究过程中，我们越来越意识到化疗/放疗后的组织病理学和盆腔CT扫描表现可能具有误导性。将患儿转诊至有盆腔RMS治疗经验的儿科中心可能有助于提高这些患儿的膀胱保留率。
