Probing for a deeper understanding of rhabdomyosarcoma: insights from complementary model systems.
Probing for a deeper understanding of rhabdomyosarcoma: insights from complementary model systems.
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DOI：
10.1038/nrc3961
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发表时间：
2015-07
期刊：
影响因子：
--
通讯作者：
Galindo RL
中科院分区：
文献类型：
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作者：
Kashi VP;Hatley ME;Galindo RL
横纹肌肉瘤（RMS）是一种间质恶性肿瘤，由肿瘤性原始前体细胞组成，表现出肌源性分化的组织学特征。尽管密集的常规多模式治疗，高危RMS患者通常患有侵袭性疾病。缺乏针对RMS的定向治疗强调了进一步揭示疾病分子基础的必要性。在这篇综述中，我们讨论了目前可用于探测新RMS靶向发病机制的模型系统的显着进展，以及增强RMS治疗和改善临床结局的可能性。
Rhabdomyosarcoma (RMS) is a mesenchymal malignancy composed of neoplastic primitive precursor cells that exhibit histological features of myogenic differentiation. Despite intensive conventional multimodal therapy, patients with high-risk RMS typically suffer from aggressive disease. The lack of directed therapies against RMS emphasizes the need to further uncover the molecular underpinnings of the disease. In this Review, we discuss the notable advances in the model systems now available to probe for new RMS-targetable pathogenetic mechanisms, and the possibilities for enhanced RMS therapeutics and improved clinical outcomes.