儿童和青少年滑膜肉瘤的生存率及影响预后的因素

E. E. Nadi, H. Hafez, A. Younes, Gehad Ahmed, M. Zaghloul, H. Taha, Naglaa Elkinaai, Maeha ELwakeel, Salma Elmenawi, Elham Khalid, S. Fadel, Soha Ahmed
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前言:探讨儿童和青少年滑膜肉瘤的临床和病理特征对其预后的影响。方法:回顾性分析2007年7月至2016年12月在埃及儿童癌症医院57,357 (CCHE)诊断为滑膜肉瘤并治疗的18岁以下患者。我们回顾了临床特征、病理信息、治疗方式和生存数据。结果:我们纳入31例患者,诊断时中位年龄为14.8岁。10例(58.8%)患者可以进行首次手术切除,5例(29.4%)患者在术前化疗反应后进行手术切除。2例患者肿瘤不可切除,化疗无效,仅局部对照放疗。仅辅助放疗2例,化疗不局部放疗5例,双管齐下10例。估计全组5年总生存率和无失败生存率分别为84.4%±7.2%和63.8%±9.7%,5年OS和FFS与患者年龄、肿瘤大小、不同病理类型、病变部位和局部控制方法的相关性进行研究。在肿瘤大小小于5 cm的患者中,与大于5 cm的患者相比,OS为(100%),而在初始或化疗后进行完全手术切除的患者中,OS为(79.3±9.3),对于肉眼或显微镜下残留的患者,OS为66.7%,而55.6% (p值= .38)。此外,3年无失败生存率为75%,小于或等于5 cm和大于5 cm的患者分别为56.3% (p值= .3)。结论:增加患者数量以增加样本量来评估肿瘤大小和手术完全切除是我们研究中重要的预后因素,我们发现它们在统计学上不显著。术前化疗可以帮助患者延迟切除最初提出了不可切除的肿瘤
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Survival and factors affecting the outcome of synovial sarcoma in children and adolescents single institute experience
Introduction: To check the impact of the clinical and pathologic features at diagnosis and treatment given, on the outcome of synovial sarcoma in children and adolescents.Methods: Retrospective analysis of patients below 18 years old diagnosed by synovial sarcoma and treated at Children CancerHospital Egypt 57,357 (CCHE) between July 2007 and December 2016. We reviewed Clinical characteristics, pathological information, treatment modalities and survival data.Results: We included thirty one patients with median age at diagnosis was 14.8 years. Initial surgical excision was possible in 10 patients (58.8%) while 5 (29.4%) patients underwent surgical excision after response to preoperative chemotherapy. Two patients had unresectable tumor, showed no response to chemotherapy and received radiotherapy as the only local control therapy. Adjuvant radiotherapy only was given in 2 patients and 5 patients received chemotherapy without local radiotherapy and 10 patients received both modalities. The estimated 5-year overall survival and failure free survival rates for the entire group were 84.4% ± 7.2% and 63.8% ± 9.7% respectively, the 5-year OS and FFS were studied in correlation to age of patient, tumor size, different pathological types, site of the lesion and methods of local control. In patients with tumor size below 5 cm when compared to size more than 5 cm with OS was (100%) in comparison to (79.3 ± 9.3) who underwent complete surgical excision either initial or post chemotherapy as it was 66.7% versus 55.6% for those with gross or microscopic residual (p-value = .38). Also, the 3-year failure free survival was 75% versus 56.3% for those smaller than or equal 5 cm and those larger than 5 cmrespectively (p-value = .3).Conclusion: Increasing number of patients to increase sample size to assess tumor size and complete surgical excision as important prognostic factors as in our study, we found them statistically insignificant. Preoperative chemotherapy can help for delayed excision in patients presented initially with unresectable tumors
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