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.

肺泡软部肉瘤是一种间叶性恶性肿瘤，以ASPSCR1和TFE3重排为特征和组织学上独特的假肺泡模式。尽管肺泡软部分肉瘤病程缓慢，但由于晚期远处转移，其长期预后较差。目前，尚未发现肺泡软部分肉瘤的治愈性治疗方法，因此长期以来一直需要一种新的治疗策略。患者来源的细胞系是基础和临床前研究的重要工具。然而，文献中很少报道来自肺泡软组织肉瘤的细胞系，因为它是一种极为罕见的恶性肿瘤，仅占所有软组织肉瘤的不到 1%。本研究旨在建立一种新型的肺泡软部肉瘤细胞系。利用手术切除的肺泡软组织肉瘤肿瘤组织，我们成功建立了细胞系，并将其命名为NCC-ASPS1-C1。ASPSCR1 - TFE3融合基因并​​表现出缓慢的生长和球状体的形成。另一方面，NCC-ASPS1-C1 没有表现出入侵的能力。我们筛选了 195 种抗癌药物的抗增殖作用，包括食品和药物管理局批准的抗癌药物。我们发现 MET 抑制剂 tivantinib 和多激酶抑制剂 orantinib 抑制 NCC-ASPS1-C1 细胞的增殖。这些药物的临床应用和抗肿瘤作用的分子机制值得进一步研究，NCC-ASPS1-C1细胞将成为肺泡软部分肉瘤体外研究的有用工具。