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Establishment and characterization of a novel patient-derived Ewing sarcoma cell line, NCC-ES2-C1

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Abstract

Ewing sarcoma (ES) is a small round cell sarcoma that is characterized by the unique gene translocation EWSR1–FLI1. It is the second most common primary bone and soft tissue malignancy in children and adolescents. It constitutes 10–15% of all bone sarcomas and is highly aggressive and rapidly recurring. Although intensive treatments have improved the clinical outcome of ES patients, 20–25% of them exhibit metastases during diagnosis. Thus, the prognoses of these patients remain poor. Cell lines are pivotal resources to investigate the molecular background of disease progression and to develop novel therapeutic modalities. In this study, we established and characterized a novel ES cell line, NCC-ES2-C1. The presence of the EWSR1–FLI1 fusion gene in these cells was confirmed in the NCC-ES2-C1 cells. Furthermore, these cells exhibited constant proliferation, and invasion, but did not form tumors in mice. We screened the anti-tumor effects of 214 anti-cancer drugs in NCC-ES2-C1 cells and found that the drugs which effectively reduced the proliferation of NCC-ES2-C1 cells. We concluded that NCC-ES2-C1 cells are a useful resource to study functions of the EWSR1–FLI1 fusion gene, investigate phenotypic changes caused by genes and proteins, and evaluate the anti-tumor effects of novel drugs.

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Acknowledgements

We would like to thank the Tochigi Cancer Center operating room’s nurse team and the secretary of the Medical Office for their assistance in processing and transporting the samples. We also appreciate the technical assistance provided by Mrs. Y. Kuwata (Division of Rare Cancer Research) and Mr. T. Kondo (Hokkaido University Medical School). We would like to thank Editage (www.editage.jp) for providing English language editing services and for their constructive comments on this manuscript. This study was technically supported by the Fundamental Innovative Oncology Core of the National Cancer Center.

Funding

This research was supported by the Japan Agency for Medical Research and Development (Grant number: 20ck0106537h0002).

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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, Yooksil Sin, Ryuto Tsuchiya, Takuya Ono, Taro Akiyama & Tadashi Kondo

  2. Division of Musculoskeletal Oncology and Orthopaedics Surgery, Tochigi Cancer Center, 4-9-13 Yohnan, Utsunomiya, Tochigi, 320-0834, Japan

    Rumi Nakagawa, Satoshi Kamio & Kazutaka Kikuta

  3. Division of Diagnostic Pathology, Tochigi Cancer Center, 4-9-13 Yohnan, Utsunomiya, Tochigi, 320-0834, Japan

    Kaoru Hirabayashi

  4. Division of Hepato-Biliary-Pancreatic Surgery, Tochigi Cancer Center, 4-9-13 Yohnan, Utsunomiya, Tochigi, 320-0834, Japan

    Iwao Ozawa

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  1. Yuki Yoshimatsu
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Correspondence to Tadashi Kondo.

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Ethical approval

The ethical committees of the Tochigi Cancer Center and the National Cancer Center approved the use of clinical materials for this study.

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Written informed consent for publication was provided by the patients.

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Supplementary Information

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13577_2022_701_MOESM1_ESM.tif

Supplementary file1 Supplementary Fig. 1. Short tandem repeat patterns in the NCC-ES2-C1 cell line and the original tumor tissue. (A) Short tandem repeat patterns in NCC-ES2-C1 cells. (B) Short tandem repeat patterns in the original tumor tissue. (TIF 257 KB)

Supplementary file2 Supplementary Table 1. List of the anti-cancer drugs used in this study. (XLSX 22 KB)

13577_2022_701_MOESM3_ESM.xlsx

Supplementary file3 Supplementary Table 2. Viability (%) of NCC-ES2-C1 cells after treatment with 214 anti-cancer drugs (XLSX 26 KB)

13577_2022_701_MOESM4_ESM.xlsx

Supplementary file4 Supplementary Table 3. List of Ewing sarcoma cell lines recorded in the cell line database Cellosaurus (XLSX 19 KB)

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Yoshimatsu, Y., Noguchi, R., Sin, Y. et al. Establishment and characterization of a novel patient-derived Ewing sarcoma cell line, NCC-ES2-C1. Human Cell 35, 1262–1269 (2022). https://doi.org/10.1007/s13577-022-00701-9

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