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Establishment and characterization of NCC-DFSP3-C1: a novel patient-derived dermatofibrosarcoma protuberans cell line

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Abstract

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.

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Acknowledgements

We thank Drs. F. Nakatani, S. Fukushima, T. Komatsubara, M. Saito, C. Sato (Department of Musculoskeletal Oncology), and Drs. T. Shibayama, and H. Tanaka (Department of Diagnostic Pathology), National Cancer Center Hospital, for sampling tumor tissue specimens from surgically resected materials. We also appreciate the technical assistance by Mr. K. Tanoue, T. Ono (Division of Rare Cancer Research). We would also like to thank Editage (www.editage.jp) for their help with English language editing and their constructive comments on the manuscript. This research was technically assisted by the Fundamental Innovative Oncology Core in the National Cancer Center (Tokyo, Japan).

Funding

This research was financially supported by the National Cancer Center Research and Development Fund (grant nos. 29-A-2) (Tokyo, Japan).

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Correspondence to Tadashi Kondo.

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Yoshimatsu, Y., Noguchi, R., Tsuchiya, R. et al. Establishment and characterization of NCC-DFSP3-C1: a novel patient-derived dermatofibrosarcoma protuberans cell line. Human Cell 33, 894–903 (2020). https://doi.org/10.1007/s13577-020-00365-3

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