CIC-DUX4 sarcoma (CDS), an aggressive soft tissue sarcoma, is characterized by a CIC and DUX4 rearrangement. It has a dismal clinical course and high metastatic rate and shows chemotherapy resistance; therefore, a novel therapeutic strategy is required. Patient-derived cell lines are indispensable tools for basic and preclinical research. However, only a few patient-derived CDS cell lines have been currently reported. Therefore, in this study, we aimed to establish and characterize a novel cell line of CDS. We successfully established the NCC-CDS2-C1 cell line by using surgically resected tumor tissue from a patient with CDS. The NCC-CDS2-C1 cells harbored a CIC-DUX4 fusion gene without insertion and exhibited rapid growth, spheroid formation, and invasion. We screened the antiproliferative effects of small anticancer agent compounds, which included FDA-approved anticancer drugs, on NCC-CDS2-C1 cells in comparison with those on the two previously reported patient-derived CDS cell lines, NCC-CDS1-X1-C1 and NCC-CDS1-X3-C1. The response profile of NCC-CDS2-C1 was similar to but distinct from those of the other cell lines for the small anticancer agent compounds. Therefore, we conclude that the NCC-CDS2-C1 cell line will be a useful tool for basic and preclinical studies of CDS.

中文翻译：

---

NCC-CDS2-C1的建立和表征：一种新的患者来源的CIC-DUX4肉瘤细胞系。

CIC - DUX4肉瘤 (CDS) 是一种侵袭性软组织肉瘤，以CIC和DUX4重排为特征。临床病程惨淡，转移率高，对化疗耐药；因此，需要一种新的治疗策略。患者来源的细胞系是基础和临床前研究不可或缺的工具。然而，目前仅报道了少数患者来源的 CDS 细胞系。因此，在本研究中，我们旨在建立和表征一种新的 CDS 细胞系。我们通过使用来自 CDS 患者的手术切除的肿瘤组织成功地建立了 NCC-CDS2-C1 细胞系。NCC-CDS2-C1 细胞含有CIC - DUX4融合基因没有插入，并表现出快速生长、球状体形成和侵袭。我们筛选了小型抗癌剂化合物（包括 FDA 批准的抗癌药物）对 NCC-CDS2-C1 细胞的抗增殖作用，并与之前报道的两种患者来源的 CDS 细胞系 NCC-CDS1-X1-C1 和NCC-CDS1-X3-C1。NCC-CDS2-C1 的反应谱类似于但不同于其他细胞系对小抗癌剂化合物的反应谱。因此，我们得出结论，NCC-CDS2-C1 细胞系将​​成为 CDS 基础和临床前研究的有用工具。