Dedifferentiated liposarcoma (DDLPS) is one of the four subtypes of liposarcomas; it is characterized by the amplification of the 12q13-15 region, which includes MDM2 and CDK4 genes. DDLPS has an extremely high local recurrence rate and is refractory to chemotherapy and radiation, which leads to poor prognosis. Therefore, a novel therapeutic strategy should be urgently established for improving the prognosis of DDLPS. Although patient-derived cell lines are important tools for basic research, there are no DDLPS cell lines available from public cell banks. Here, we report the establishment of a novel DDLPS cell line. Using the surgically resected tumor tissue from a patient with DDLPS, we established a cell line and named it NCC-DDLPS1-C1. The NCC-DDLPS1-C1 cells contained 12q13-15, 1p32, and 1q23 amplicons and highly expressed MDM2 and CDK4 proteins. NCC-DDLPS-C1 cells exhibited constant growth, spheroid formation, aggressive invasion, and tumorigenesis in mice. By screening a drug library, we identified that the proteasome inhibitor, bortezomib, had inhibitory effects on the proliferation of NCC-DDLPS1-C1 cells. We concluded that the NCC-DDLPS1-C1 cell line may serve as a useful tool for basic and pre-clinical studies of DDLPS.

去分化脂肪肉瘤（DDLPS）是脂肪肉瘤的四种亚型之一。它的特点是扩增了12q13-15区域，其中包括MDM2和CDK4基因。DDLPS具有极高的局部复发率，并且对化学疗法和放射线难以治疗，从而导致不良预后。因此，迫切需要建立一种新的治疗策略以改善DDLPS的预后。尽管患者来源的细胞系是基础研究的重要工具，但公共细胞库尚无DDLPS细胞系。在这里，我们报告新型DDLPS细胞系的建立。使用来自DDLPS患者的手术切除的肿瘤组织，我们建立了一个细胞系，并将其命名为NCC-DDLPS1-C1。NCC-DDLPS1-C1细胞包含12q13-15、1p32和1q23扩增子以及高表达的MDM2和CDK4蛋白。NCC-DDLPS-C1细胞在小鼠中表现出恒定的生长，球状体形成，侵袭性侵袭和肿瘤发生。通过筛选药物库，我们发现蛋白酶体抑制剂硼替佐米对NCC-DDLPS1-C1细胞的增殖具有抑制作用。我们得出的结论是，NCC-DDLPS1-C1细胞系可作为DDLPS基础和临床前研究的有用工具。