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Establishment and characterization of NCC-CDS2-C1: a novel patient-derived cell line of CIC-DUX4 sarcoma

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Abstract

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.

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Acknowledgements

We thank Drs. Y. Minami, K. Shimizu, T. Mori, T. Uehara M. Sugawara, Y. Araki, and T. Hirose from the Division of Musculoskeletal Oncology, National Cancer Center Hospital, for sampling tumor tissue specimens from surgically resected materials. We appreciate the technical assistance provided by Mr. T. Ono and K. Tanoue. We would like to thank Editage (www.editage.jp) for English-language editing and their constructive comments on the manuscript. This research was financially supported by the National Cancer Center Research and Development Fund (Grant no. 29-A-2).

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Authors and Affiliations

  1. Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Yuki Yoshimatsu, Rei Noguchi, Ryuto Tsuchiya, Fusako Kito, Akane Sei & Tadashi Kondo

  2. Department of Orthopaedic Surgery, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670, Japan

    Ryuto Tsuchiya

  3. Division of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Jun Sugaya, Makoto Nakagawa, Shintaro Iwata & Akira Kawai

  4. Department of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Akihiko Yoshida

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  1. Yuki Yoshimatsu
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  2. Rei Noguchi
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  3. Ryuto Tsuchiya
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  4. Fusako Kito
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  5. Akane Sei
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  6. Jun Sugaya
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  8. Akihiko Yoshida
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  10. Akira Kawai
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  11. Tadashi Kondo
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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-CDS2-C1: a novel patient-derived cell line of CIC-DUX4 sarcoma. Human Cell 33, 427–436 (2020). https://doi.org/10.1007/s13577-019-00312-x

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