This paper provides insights into the use of Proton Beam Therapy (PBT) in pediatric patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). NRSTS are a heterogeneous group of rare and aggressive mesenchymal extraskeletal tumors, presenting complex and challenging clinical management scenarios. The overall survival rate for patients with NRSTS is around 70%, but the outcome is strictly related to the presence of various variables, such as the histological subtype, grade of malignancy and tumor stage at diagnosis. Multimodal therapy is typically considered the preferred treatment for high-grade NRSTS. Radiotherapy plays a key role in the treatment of children and adolescents with NRSTS. However, the potential for radiation-induced side effects partially limits its use. Therefore, PBT represents a very suitable therapeutic option for these patients. The unique depth-dose characteristics of protons can be leveraged to minimize doses to healthy tissue significantly, potentially allowing for increased tumor doses and enhanced preservation of surrounding tissues. These benefits suggest that PBT may improve local control while reducing toxicity and improving quality of life. While clear evidence of therapeutic superiority of PBT over other modern photon techniques in NRSTS is still lacking—partly due to the limited data available—PBT can be an excellent treatment option for young patients with these tumors. A dedicated international comprehensive collaborative approach is essential to better define its role within the multidisciplinary management of NRSTS. Shared guidelines for PBT indications—based on the patient’s age, estimated outcome, and tumor location—and centralization in high-level referral centers are needed to optimize the use of resources, since access to PBT remains a challenge due to the limited number of available proton therapy facilities.
本文探讨质子束疗法在儿童非横纹肌肉瘤软组织肉瘤治疗中的应用。NRSTS是一组异质性罕见侵袭性间叶组织来源的骨外肿瘤，其临床管理复杂且极具挑战性。NRSTS患者总体生存率约为70%，但预后与多种变量密切相关，包括组织学亚型、恶性程度分级及诊断时的肿瘤分期。多模式治疗通常被认为是高级别NRSTS的首选方案。放射治疗在儿童及青少年NRSTS治疗中具有关键作用，但辐射诱发副作用的潜在风险在一定程度上限制了其应用。因此，PBT成为这类患者非常适宜的治疗选择。质子独特的深度-剂量特性可显著降低对健康组织的辐射剂量，从而可能实现肿瘤剂量提升与周围组织保护的双重优化。这些优势表明PBT在改善局部控制的同时，有望降低毒性反应并提升生活质量。尽管目前尚缺乏PBT优于其他现代光子技术的明确证据（部分归因于可用数据有限），但对于罹患此类肿瘤的年轻患者，PBT仍是卓越的治疗选择。为在NRSTS多学科治疗体系中准确定位PBT的作用，建立国际化的综合协作机制至关重要。鉴于质子治疗设施数量有限导致的可及性挑战，需要基于患者年龄、预估预后及肿瘤部位制定统一的PBT适应证指南，并在高水平转诊中心实现集中化治疗，以优化资源配置。
Proton Therapy in Non-Rhabdomyosarcoma Soft Tissue Sarcomas of Children and Adolescents
肿瘤(癌症)患者之家