Myxofibrosarcoma (MFS) is among the most aggressive and complex sarcoma types that require novel therapeutic approaches for improved clinical outcomes. MFS displays highly complex karyotypes, and frequent alterations in p53 signaling and cell cycle checkpoint genes as well as loss-of-function mutations in NF1 and PTEN have been reported. The effects of radiotherapy and chemotherapy on MFS are limited, and complete surgical resection is the only curative treatment. Thus, the development of novel therapeutic strategies for MFS has long been long desired for MFS. Patient-derived cell lines are an essential tool for basic and translational research in oncology. However, public cell banks provide only a limited number of MFS cell lines. In this study, we aimed to develop a novel patient-derived MFS cell line, which was established from the primary tumor tissue of a 71-year-old male patient with MFS and was named NCC-MFS2-C1. A single-nucleotide polymorphism assay revealed that NCC-MFS2-C1 cells exhibited gain and loss of genetic loci. NCC-MFS2-C1 cells were maintained as a monolayer culture for over 24 passages for 10 months. The cells exhibited spindle-like morphology, continuous growth, and capacity for spheroid formation and invasion. Screening of 213 anticancer agents revealed that bortezomib, gemcitabine, romidepsin, and topotecan at low concentrations inhibited the proliferation of NCC-MFS2-C1 cells. In conclusion, we established a novel MFS cell line, NCC-MFS2-C1, which can be used for studying the molecular mechanisms underlying tumor development and for the in vitro screening of anti-cancer drugs.

粘膜原纤维肉瘤（MFS）是最具攻击性和复杂性的肉瘤类型之一，需要新颖的治疗方法来改善临床疗效。MFS显示高度复杂的核型，p53信号和细胞周期检查点基因的频繁变化以及NF1 和 PTEN的功能丧失突变 已经报道。放射疗法和化学疗法对MFS的作用是有限的，完全的手术切除是唯一的治疗方法。因此，长期以来一直期望为MFS开发新的治疗策略。患者来源的细胞系是肿瘤学基础和转化研究的重要工具。但是，公共细胞库仅提供有限数量的MFS细胞系。在这项研究中，我们旨在开发一种新的患者源性MFS细胞系，该细胞系是从一名71岁男性MFS患者的原发肿瘤组织中建立的，并命名为NCC-MFS2-C1。单核苷酸多态性分析表明，NCC-MFS2-C1细胞表现出遗传基因座的增加和减少。将NCC-MFS2-C1细胞作为单层培养物维持24代以上，持续10个月。这些细胞表现出纺锤状的形态，连续的生长以及球体形成和侵袭的能力。213种抗癌药的筛选表明，低浓度的硼替佐米，吉西他滨，罗米地辛和托泊替康可以抑制NCC-MFS2-C1细胞的增殖。总之，我们建立了一种新型的MFS细胞系NCC-MFS2-C1，可用于研究肿瘤发生的分子机制和体外抗癌药物的筛选。