Roy Soumyajit, Pathy Sushmita, Mohanti Bidhu K, Chander Subhash, Biswas Ahitagni
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India.
J Pediatr Hematol Oncol. 2017 Mar;39(2):e62-e68. doi: 10.1097/MPH.0000000000000745.
The study was aimed at evaluating adherence to treatment protocol and outcome in pediatric parameningeal rhabdomyosarcoma (PM-RMS). We analyzed the characteristics, treatment administered, outcomes and patterns of failure of pediatric PM-RMS, who were treated with multimodality therapy between January 2005 and December 2013.Univariate and multivariate analysis (MVA) was completed to evaluate the impact of various prognostic factors. Thirty-seven patients were treated at our institution. Majority of them had the primary disease in paranasal sinuses (n=13). Majority of the patients belonged to group III (n=30) and stage III (n=24). The overall response rate to treatment was 52.5% (n=21). At a mean follow-up of 19.1 months, 23 patients developed disease progression. The actuarial rates of failure-free survival and overall survival (OS) at 2 years were 40% and 67.5%, respectively. Patients who received >20 weeks of intended chemotherapy schedule (P=0.02) and had complete response to first-line treatment (P=0.0004) were found to have superior failure-free survival on MVA. Complete response was the lone determinant of superior OS on MVA (P=0.006). Majority of patients with PM-RMS present with advanced stage disease. Response to first-line treatment is a significant predictor of superior progression-free survival and OS in these patients.
本研究旨在评估儿童鼻窦旁横纹肌肉瘤（PM-RMS）的治疗方案依从性及治疗结果。我们分析了2005年1月至2013年12月期间接受多模式治疗的儿童PM-RMS的特征、所接受的治疗、治疗结果及失败模式。完成单因素和多因素分析（MVA）以评估各种预后因素的影响。我们机构共治疗了37例患者。其中大多数患者的原发疾病位于鼻窦（n=13）。大多数患者属于Ⅲ组（n=30）和Ⅲ期（n=24）。治疗的总体缓解率为52.5%（n=21）。平均随访19.1个月时，23例患者出现疾病进展。2年时无失败生存率和总生存率（OS）的精算率分别为40%和67.5%。多因素分析发现，接受预期化疗疗程>20周的患者（P=0.02）以及对一线治疗有完全缓解的患者（P=0.0004）无失败生存率更高。多因素分析显示，完全缓解是总生存率更高的唯一决定因素（P=0.006）。大多数PM-RMS患者就诊时已处于疾病晚期。对一线治疗的反应是这些患者无进展生存期和总生存期更长的重要预测因素。
