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Establishment and characterization of NCC-ASPS1-C1: a novel patient-derived cell line of alveolar soft-part sarcoma

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Abstract

Alveolar soft-part sarcoma is a mesenchymal malignancy characterized by the rearrangement of ASPSCR1 and TFE3 and a histologically distinctive pseudoalveolar pattern. Although alveolar soft-part sarcoma takes an indolent course, its long-term prognosis is poor because of late distant metastases. Currently, curative treatments have not been found for alveolar soft-part sarcoma, and hence, a novel therapeutic strategy has long been required. Patient-derived cell lines comprise an important tool for basic and preclinical research. However, few cell lines from alveolar soft-part sarcoma have been reported in the literature because it is an extremely rare malignancy, accounting for less than 1% of all soft-tissue sarcomas. This study aimed to establish a novel alveolar soft-part sarcoma cell line. Using surgically-resected tumor tissue of alveolar soft-part sarcoma, we successfully established a cell line and named it NCC-ASPS1-C1. The NCC-ASPS1-C1 cells harbored an ASPSCR1-TFE3 fusion gene and exhibited slow growth, and spheroid formation. On the other hand, NCC-ASPS1-C1 did not show the capability of invasion. We screened the antiproliferative effects of 195 anticancer agents, including Food and Drug Administration-approved anticancer drugs. We found that the MET inhibitor tivantinib and multi-kinase inhibitor orantinib inhibited the proliferation of NCC-ASPS1-C1 cells. The clinical utility and molecular mechanisms of antitumor effects of these drugs are worth investigating in the further studies, and NCC-ASPS1-C1 cells will be a useful tool for the in vitro study of alveolar soft-part sarcoma.

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Acknowledgements

We thank Drs. F Nakatani, E Kobayashi, S Iwata, M Nakagawa, T Komatsubara, M Saito, and C Sato (Division of Musculoskeletal Oncology, National Cancer Center Hospital), as well as Drs. T Shibayama and H Tanaka (Department of Diagnosis Pathology, National Cancer Center Hospital), for sampling tumor tissue specimens from surgically resected materials. We appreciate the technical assistance of Mr. T Ono and K Tanoue (Division of Rare Cancer Research, National Cancer Center Institute). We appreciate the technical support by Ms. Yurika Shiotani, Mr. Naoaki Uchiya, and Dr. Toshio Imai (Central Animal Division, National Cancer Center Research Institute). We would like to thank Editage (www.editage.jp) for English-language editing and for their constructive comments on the manuscript. This research was financially supported by the National Cancer Center Research and Development Fund (grant nos. 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, 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, Suguru Fukushima & Akira Kawai

  4. Department of Diagnosis 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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Correspondence to Tadashi Kondo.

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The ethical committee of the National Cancer Center approved the use of clinical materials for this study with the approval number 2004-050.

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Yoshimatsu, Y., Noguchi, R., Tsuchiya, R. et al. Establishment and characterization of NCC-ASPS1-C1: a novel patient-derived cell line of alveolar soft-part sarcoma. Human Cell 33, 1302–1310 (2020). https://doi.org/10.1007/s13577-020-00382-2

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