背景:眼眶横纹肌肉瘤(RMS),儿童时期最常见的原发性恶性眼眶肿瘤,由于其遗传基础和异常的细胞增殖,在管理方面提出了独特的挑战。管理已经从手术切除发展到多模式方法,包括手术,放射治疗,和化疗。这篇综述探讨了流行病学的趋势,病理生理学见解,和治疗进展,以描绘最佳治疗策略。
方法:在截至2024年3月4日的PubMed和Scopus数据库中使用与轨道RMS相关的关键词进行了文献检索。研究是根据预定义的标准选择的,专注于临床表现,诊断方式,管理策略,和结果。数据提取和分析由两名评审员独立进行,根据牛津循证医学中心2011年指南和乔安娜·布里格斯研究所检查表进行质量评估。
结果:这篇综述综合了17项研究的数据,突出人口模式,临床特征,分期,和治疗方法。大多数病人是男性和白人,早期诊断的比例更高。胚胎亚型是最常见的,治疗已经转向放射治疗和联合治疗方式。在轨道RMS中,在治疗中应用了几种方式。有放射治疗,手术干预,和化疗。最近的研究更多的重点是眼眶骨发育和面部对称性的疾病的长期结果。
结论:轨道RMS的管理有了很大的发展,目前强调多模式治疗策略。未来的研究应集中在优化治疗方法,以平衡有效的肿瘤控制与保留眼眶功能和美学。
BACKGROUND: Orbital
rhabdomyosarcoma (RMS), the most common primary malignant orbital tumor in childhood, presents unique challenges in management due to its genetic basis and abnormal cellular proliferation. Management has evolved from surgical excision to multimodal approaches, including surgery, radiotherapy, and chemotherapy. This
review explores trends in epidemiology, pathophysiological insights, and treatment evolution to delineate optimal therapeutic strategies.
METHODS: A literature search was conducted across PubMed and Scopus databases up to March 4, 2024, using keywords related to orbital RMS. Studies were selected based on predefined criteria, focusing on clinical presentation, diagnostic modalities, management strategies, and outcomes. Data extraction and analysis were performed independently by 2 reviewers, with quality assessment based on the Oxford Center for Evidence-Based Medicine 2011 guidelines and Joanna Briggs Institute checklists.
RESULTS: The
review synthesized data from 17 studies, highlighting demographic patterns, clinical characteristics, staging, and treatment approaches. Most patients were male and white, with a higher proportion of early-stages diagnoses. The embryonal subtype was the most common, and treatment has shifted toward radiation therapy and combined modalities. In orbital RMS, several modalities are applied in treatment. There are radiotherapy, surgical interventions, and chemotherapy. Recent studies put more emphasis on the long-term outcome of the disease about orbital bone development and facial symmetry.
CONCLUSIONS: Management of orbital RMS has evolved significantly, with a current emphasis on multimodal treatment strategies. Future research should focus on optimizing therapeutic approaches to balance effective tumor control with the preservation of orbital function and esthetics.