The role of radiotherapy in multimodal treatment of non-rhabdomyosarcoma soft tissue sarcomas in children: A real life report from a tertiary center.

Alper Kahvecioglu, Sezin Yuce Sari, Ecem Yigit, Melis Gultekin, Kemal Kosemehmetoglu, Diclehan Orhan, Nilgun Kurucu, Mustafa Tezer Kutluk, Ferah Yildiz
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Abstract

Objective: The current treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is a multimodal risk-based approach. Today, smaller fields and lower doses of radiotherapy (RT) have become standard. In this study, it was aimed to evaluate the treatment outcomes and toxicity profile in children with NRSTS that received RT as a part of multimodal therapy.

Methods: Twenty-nine patients with pediatric NRSTS treated with neoadjuvant or adjuvant RT between 1998 and 2022 were evaluated retrospectively. Kaplan-Meier method was used for survival analyses.

Results: Median follow-up was 36 months (range, 6-291 months). The median neoadjuvant and adjuvant RT doses were 50 Gy (range, 45-66 Gy) and 54 Gy (45-66 Gy), respectively. During follow-up, six (21%) patients developed a local recurrence and 10 (35%) had distant metastasis. The 5-year local control, overall survival (OS), local recurrence-free survival, and distant metastasis-free survival rate was 79%, 67%, 59%, and 61%, respectively. In multivariate analysis, a ≤5-cm tumor, gross tumor resection, Children's Oncology Group (COG) low-risk group, and absence of neoadjuvant chemotherapy were independent favorable prognostic factors for OS. Severe (≥ grade 3) late toxicity was observed in 6 (20%) patients.

Conclusions: RT is a crucial component in the multimodal risk-based treatment approach for pediatric NRSTS. However, late toxicity rates are still high and should be improved. Patients with a ≤5-cm tumor, COG low-risk group and treated with gross tumor resection have increased survival rates.

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放疗在儿童非横纹肌肉瘤软组织肉瘤多模式治疗中的作用:来自一家三级医疗中心的真实报告。
目的:目前治疗小儿非横纹肌肉瘤软组织肉瘤(NRSTS)的方法是基于风险的多模式疗法。如今,更小范围、更低剂量的放射治疗(RT)已成为标准疗法。本研究旨在评估作为多模式疗法一部分接受RT治疗的NRSTS患儿的治疗效果和毒性概况:方法:对1998年至2022年间接受新辅助或辅助RT治疗的29例小儿NRSTS患者进行回顾性评估。结果:中位随访时间为36个月(范围在10个月内):中位随访时间为36个月(6-291个月)。新辅助和辅助RT的中位剂量分别为50 Gy(范围为45-66 Gy)和54 Gy(45-66 Gy)。随访期间,有6例(21%)患者出现局部复发,10例(35%)出现远处转移。5年局部控制率、总生存率(OS)、无局部复发生存率和无远处转移生存率分别为79%、67%、59%和61%。在多变量分析中,肿瘤≤5厘米、肿瘤大体切除、儿童肿瘤学组(COG)低风险组和未接受新辅助化疗是OS的独立有利预后因素。6例(20%)患者出现严重(≥3级)晚期毒性:结论:RT是小儿NRSTS基于风险的多模式治疗方法的重要组成部分。结论:RT 是小儿 NRSTS 基于风险的多模式治疗方法的重要组成部分,但晚期毒性仍较高,应加以改进。肿瘤≤5厘米、COG低风险组和接受肿瘤大体切除术的患者生存率更高。
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