Meta-analysis of BCOR rearranged sarcomas: challenging the therapeutic approach
Meta-analysis of BCOR rearranged sarcomas: challenging the therapeutic approach
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DOI：
10.1080/0284186x.2021.1890818
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发表时间：
2021-02-17
期刊：
影响因子：
3.1
通讯作者：
Bagos, Pantelis
中科院分区：
文献类型：
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作者：
Kyriazoglou, Anastasios;Bagos, Pantelis
BCOR重排肉瘤包括一组恶性间充质瘤，直到最近才被归类为尤文肉瘤或未分化圆细胞肉瘤。BCOR-CCNB3、BCOR-MAML3、ZC3H7B-BCOR融合基因和BCOR内串联重复(ITD)等BCOR基因突变的鉴定是鉴别诊断BCOR重排肉瘤的重要指标。由于这些肿瘤的罕见，对于临床医生应该遵循的最佳治疗算法还没有共识或指南。在此，我们对目前关于BCOR重排肉瘤治疗方法的报道进行了荟萃分析。结果从10个研究中选取57例符合条件的病例进行荟萃分析，结果发现接受Ewing方案和非Ewing导向治疗的患者的发病率比(IRR)和总生存率(OS)相似。两种方案的死亡率进一步相似(非Ewing方案为20%，Ewing方案为21.8%)。结论我们的数据支持非Ewing方案可以被认为是一种安全的方案，至少与Ewing方案相同。本研究为BCOR重排肉瘤的最佳治疗方法提供了线索。此外，本研究对尤文样肉瘤这一术语的使用提出了挑战，因为目前的文献支持BCOR重排肉瘤应该在遗传学、病理学和治疗方面有自己独特的分类。
IntroductionBCOR rearranged sarcomas comprise a group of malignant mesenchymal tumors that until recently were classified as Ewing sarcomas or as undifferentiated round cell sarcomas. The identification of alterations involving BCOR gene such as BCOR-CCNB3, BCOR-MAML3, ZC3H7B-BCOR fusion genes and BCOR internal tandem duplication (ITD) is characteristic for the differential diagnosis of BCOR rearranged sarcomas. Due to the rarity of these tumors there is no consensus or guidelines regarding the optimal therapeutic algorithm, that clinicians should follow.Patients and methodsHerein we have conducted a meta-analysis of the current reports dealing with the therapeutic approach of BCOR rearranged sarcomas.ResultsMeta-analysis of the 57 eligible cases from 10 studies resulted to similar Incidence Rate Ratio (IRR) and overall survival (OS) for patients who received Ewing protocols and non-Ewing oriented treatment. Further similar death rate was reported for both strategies (non-Ewing 20% Vs Ewing 21.8%).ConclusionOur data support that non-Ewing treatment strategy can be considered a safe option, being at least equal to Ewing protocols. The current study provides a hint toward the optimal therapeutic approach of BCOR rearranged sarcomas. Further, the present study challenges the use of the term Ewing-like sarcomas, since the current literature supports that BCOR rearranged sarcomas deserve their own distinct classification in terms of genetics, pathology and therapy.