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首页> 外文期刊>Journal of Clinical Oncology >Treatment of nonmetastatic cranial parameningeal rhabdomyosarcoma in children younger than 3 years old: results from international society of pediatric oncology studies MMT 89 and 95.
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Treatment of nonmetastatic cranial parameningeal rhabdomyosarcoma in children younger than 3 years old: results from international society of pediatric oncology studies MMT 89 and 95.

机译：3岁以下儿童的非转移性颅脑膜旁横纹肌肉瘤的治疗：国际儿童肿瘤学会MMT 89和95的结果。

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PURPOSE: To explore a strategy by which radiotherapy (RT) could be avoided in the treatment of young children with parameningeal rhabdomyosarcoma (PM RMS). PATIENTS AND METHODS: Fifty-nine children (median age, 2 years 3 months) with nonmetastatic cranial PM RMS were treated in the International Society of Pediatric Oncology MMT 89 and 95 trials between 1989 and 2003. RESULTS: Five-year EFS and OS rates were 46% and 54%, respectively, for the whole group. No standard clinical or pathologic variables had prognostic impact. Fifty (85%) of 59 patients achieved complete local control either with (n = 28) or without (n = 22) RT administered as part of their primary treatment. Nine patients (15%) did not achieve local control (four of whom had had RT), and all died. Patients who received RT had a significantly superior 5-year EFS rate compared with patients who did not receive RT (59% v 28%, respectively). Twenty-three patients (48%) experienced relapse at a median interval of 15 months. Ultimately, only seven patients (12%) were cured without RT, although this represented 32% of those who achieved local control with initial chemotherapy. CONCLUSION: Despite concerns about the late effects of its use in young children, cure of PM RMS remains unlikely without systematic use of RT. The accurate prediction of the small subset of patients who achieve local control without RT and who do not experience relapse would provide an opportunity for a minority of patients to avoid RT.
机译：目的：探讨可避免在治疗伴有脑膜旁横纹肌肉瘤（PM RMS）的幼儿中使用放射疗法的策略。患者和方法：1989年至2003年间，国际小儿肿瘤学会MMT 89和95试验治疗了59例非转移性颅内PM RMS患儿（中位年龄为2岁3个月）。结果：五年EFS和OS率整个组分别为46％和54％。没有标准的临床或病理变量对预后有影响。 59例患者中有50例（85％）在接受（n = 28）或不接受（n = 22）局部治疗的情况下达到了完全局部控制。九名患者（15％）未达到局部控制（其中四名接受了放疗），全部死亡。与未接受RT的患者相比，接受RT的患者的5年EFS率明显更高（分别为59％对28％）。 23名患者（48％）的复发间隔为15个月。最终，只有7例患者（12％）在没有放疗的情况下得以治愈，尽管这代表了通过初始化疗实现局部控制的患者的32％。结论：尽管担心其在幼儿中的后期作用，但如果不系统地使用RT，则治愈PM RMS的可能性仍然很小。对没有RT的局部控制且未经历复发的一小部分患者的准确预测，将为少数患者避免RT提供机会。

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