Dermatofibrosarcoma protuberans (DFSP) is the most common dermal sarcoma; it is characterized by the presence of the COL1A1–PDGFB translocation, which causes the constitutive activation of the platelet-derived growth factor β (PDGFB) signaling pathway. DFSP frequently exhibits local recurrence and is refractory to conventional chemotherapy. Therefore, a novel therapeutic strategy is required for improving the prognosis of DFSP. Although patient-derived cell lines are important tools for pre-clinical studies, currently, only a few such cell lines are available for DFSP in cell banks. Here, we report the establishment of a novel DFSP cell line. Using a surgically resected metastatic tumor tissue from a patient with DFSP, we established a cell line called NCC-DFSP3-C1. The NCC-DFSP3-C1 cells had a COL1A1–PDGFB translocation and retained the same copy number aberrations as the original tumor tissue. NCC-DFSP3-C1 cells exhibited constant growth, spheroid formation, and invasive ability. By screening a drug library, we identified anti-cancer agents with inhibitory effects on the proliferation of NCC-DFSP3-C1 cells; these anti-cancer agents included proteasomal, histone deacetylase, and kinase inhibitors. We concluded that the NCC-DFSP3-C1 cell line may serve as a useful tool for performing basic and pre-clinical studies on DFSP.

中文翻译：

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NCC-DFSP3-C1的建立和表征：一种新型的患者源性皮肤纤维肉瘤隆突细胞系。

隆突性皮肤皮肤肉瘤（DFSP）是最常见的皮肤肉瘤。它的特征是存在COL1A1-PDGFB易位，这会导致血小板衍生的生长因子β（PDGFB）信号传导途径的组成性激活。DFSP经常表现出局部复发，并且对常规化疗是难治的。因此，需要新的治疗策略来改善DFSP的预后。尽管患者来源的细胞系是进行临床前研究的重要工具，但目前，只有少数此类细胞系可用于DFSP细胞库中。在这里，我们报告了新型DFSP细胞系的建立。使用来自DFSP患者的手术切除的转移性肿瘤组织，我们建立了一种称为NCC-DFSP3-C1的细胞系。NCC-DFSP3-C1细胞有一个COL1A1-PDGFB易位并保留与原始肿瘤组织相同的拷贝数畸变。NCC-DFSP3-C1细胞表现出恒定的生长，球状体形成和侵袭能力。通过筛选药物库，我们确定了对NCC-DFSP3-C1细胞增殖具有抑制作用的抗癌剂；这些抗癌药包括蛋白酶体，组蛋白脱乙酰基酶和激酶抑制剂。我们得出的结论是，NCC-DFSP3-C1细胞系可以用作进行DFSP基础和临床前研究的有用工具。