Establishment and characterization of NCC-ASPS1-C1: a novel patient-derived cell line of alveolar soft-part sarcoma
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CELL LINE
Establishment and characterization of NCC‑ASPS1‑C1: a novel patient‑derived cell line of alveolar soft‑part sarcoma Yuki Yoshimatsu1 · Rei Noguchi1 · Ryuto Tsuchiya1,2 · Akane Sei1 · Jun Sugaya3 · Suguru Fukushima3 · Akihiko Yoshida4 · Akira Kawai3 · Tadashi Kondo1 Received: 16 April 2020 / Accepted: 25 May 2020 © Japan Human Cell Society and Springer Japan KK, part of Springer Nature 2020
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 longterm 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 softpart 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. Keywords Alveolar soft-part sarcoma · Patient-derived cancer model · Cell line · Orantinib
Introduction
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s13577-020-00382-2) contains supplementary material, which is available to authorized users. * Tadashi Kondo [email protected] 1
Division of Rare Cancer Research, National Cancer Center Research Institute, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan
2
Department of Orthopaedic Surgery, Graduate School of Medicine, Chiba University, 1‑8‑1, Inohana, Chuo‑ku, Chiba 260‑8670, Japan
3
Department of Musculoskeletal Oncology, National Cancer Center Hospital, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan
4
Department of Diagnosis Pathology, National Cancer Center Hospital, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan
Alveolar soft-part sarcoma (ASPS) is a mesenchymal malignancy that is characterized by the presence of the specific chromosome translocation, der(17)t(X;17) (p11.2;q25). This fusion of TFE3 tran
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