Spindle-cell/sclerosing rhabdomyosarcoma (ssRMS) is a rare subtype of rhabdomyosarcoma, characterized by unique pathological features. Although distinctive molecular backgrounds such as frequent mutations in MyoD1 have been reported, optimized therapy has not been fully developed, and further investigations are required. Patient-derived cancer models are critical tools for basic and pre-clinical studies. However, there is no model for ssRMS. Thus, this study aimed to develop a novel cell line from the tumor tissue of a patient with ssRMS. Using surgically resected tissue, we successfully established this cell line, named NCC-ssRMS1-C1. These cells exhibited spindle-shape morphology, consistent with the pathological observations of the original tumor tissue. Genetic studies demonstrated that NCC-ssRMS1-C1 cells retained original copy number alterations and the typical point mutation in MyoD1. Malignant phenotypes such as proliferation, spheroid formation, and invasion were confirmed in vitro by studying NCC-ssRMS1-C1 cells. Upon screening an anti-cancer agent library, sensitivity to conventional chemotherapeutic agents such as actinomycin D was revealed. We conclude that the NCC-ssRMS1-C1 cell line will be a useful resource for basic and pre-clinical studies.

中文翻译：

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NCC-ssRMS1-C1的建立和表征：一种新型的患者衍生的梭形细胞/硬化性横纹肌肉瘤细胞系。

梭形细胞/硬化性横纹肌肉瘤（ssRMS）是横纹肌肉瘤的一种罕见亚型，具有独特的病理特征。尽管独特的分子背景，例如MyoD1中的频繁突变据报道，优化疗法尚未完全开发，需要进一步研究。患者来源的癌症模型是基础和临床前研究的关键工具。但是，没有ssRMS的模型。因此，该研究旨在从患有ssRMS的患者的肿瘤组织中开发新型细胞系。使用手术切除的组织，我们成功建立了该细胞系，命名为NCC-ssRMS1-C1。这些细胞表现出纺锤形的形态，与原始肿瘤组织的病理观察一致。遗传研究表明，NCC-ssRMS1-C1细胞保留了原始拷贝数变化和MyoD1中的典型点突变。通过研究NCC-ssRMS1-C1细胞，在体外证实了恶性表型，例如增殖，球体形成和侵袭。在筛选抗癌剂文库时，显示出对常规化学治疗剂如放线菌素D的敏感性。我们得出结论，NCC-ssRMS1-C1细胞系将是基础和临床前研究的有用资源。