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Establishment and characterization of NCC-ssRMS1-C1: a novel patient-derived spindle-cell/sclerosing rhabdomyosarcoma cell line

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Abstract

Spindle-cell/sclerosing rhabdomyosarcoma (ssRMS) is a rare subtype of rhabdomyosarcoma, characterized by unique pathological features. Although distinctive molecular backgrounds such as frequent mutations in MyoD1 have been reported, optimized therapy has not been fully developed, and further investigations are required. Patient-derived cancer models are critical tools for basic and pre-clinical studies. However, there is no model for ssRMS. Thus, this study aimed to develop a novel cell line from the tumor tissue of a patient with ssRMS. Using surgically resected tissue, we successfully established this cell line, named NCC-ssRMS1-C1. These cells exhibited spindle-shape morphology, consistent with the pathological observations of the original tumor tissue. Genetic studies demonstrated that NCC-ssRMS1-C1 cells retained original copy number alterations and the typical point mutation in MyoD1. Malignant phenotypes such as proliferation, spheroid formation, and invasion were confirmed in vitro by studying NCC-ssRMS1-C1 cells. Upon screening an anti-cancer agent library, sensitivity to conventional chemotherapeutic agents such as actinomycin D was revealed. We conclude that the NCC-ssRMS1-C1 cell line will be a useful resource for basic and pre-clinical studies.

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Acknowledgements

We thank Drs. F Nakatani, E Kobayashi, S Fukushima, M Nakagawa, T Komatsubara, M Saito, C Sato (Department of Musculoskeletal Oncology, National Cancer Center Hospital), T Shibayama, and H Tanaka (Department of Diagnostic Pathology), for sampling tumor tissue specimens from surgically resected materials. We would like to thank Editage (www.editage.jp) for their assistance with English-language editing and their constructive comments regarding the manuscript. This research was financially supported by the National Cancer Center Research and Development Fund (Grant Nos. 29-A-2) and technically assisted by the Fundamental Innovative Oncology Core in the National Cancer Center.

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

    Jun Sugaya, Shintaro Iwata & Akira Kawai

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

    Masanaka Sugiyama

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

    Akihiko Yoshida

Authors
  1. Yuki Yoshimatsu
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  2. Rei Noguchi
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  3. Ryuto Tsuchiya
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  4. Akane Sei
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  5. Jun Sugaya
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  7. Masanaka Sugiyama
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  8. Akihiko Yoshida
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  9. Akira Kawai
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  10. Tadashi Kondo
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Contributions

YY and TK designed the study, and wrote the manuscript. RN, RT, and AS contributed to the experiments and interpretation of data, and assisted in the preparation of the manuscript. JS, SI, MS, AY, and AK have contributed to clinical samples and data collection, and the preparation of manuscript. AY contributed to the pathological diagnosis, and the preparation of manuscript. All authors critically reviewed the manuscript, approved the final version of the manuscript, and agree to be accountable for all aspects of the work in ensuring that questions related the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Corresponding author

Correspondence to Tadashi Kondo.

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The authors declare that they have no conflict of interest.

Research involving human participants

The use of clinical materials for this study was approved by the ethical committee of the National Cancer Center (2004-050).

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The written informed consent was obtained from the donor patient.

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Yoshimatsu, Y., Noguchi, R., Tsuchiya, R. et al. Establishment and characterization of NCC-ssRMS1-C1: a novel patient-derived spindle-cell/sclerosing rhabdomyosarcoma cell line. Human Cell 33, 886–893 (2020). https://doi.org/10.1007/s13577-020-00359-1

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  • DOI: https://doi.org/10.1007/s13577-020-00359-1

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