Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Division of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Hum Cell. 2021 Nov;34(6):1919-1928. doi: 10.1007/s13577-021-00612-1. Epub 2021 Sep 17.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma genetically characterized by the presence of the FUS-CREB3L2 gene fusion. While LGFMS exhibits indolent features during its early stages, the rates of recurrence, metastasis, and death from the disease are high. Presently, the role of FUS-CREB3L2 gene fusions in the unique features of LGFMS is not clear, and there is no modality to improve the clinical outcomes of patients with LGFMS; thus, extensive studies on LGFMS are required. Patient-derived cancer cell lines are critical tools for cancer research. However, no cell line has been established for LGFMS. Here, we aimed to develop a novel cell line for LGFMS and successfully established it using surgically resected tumor tissues. The cells, named NCC-LGFMS1-C1, possessed the same fusion genes as their original tumor and visible copy number variations. The cells had a fibroblastic appearance, formed spheroids when they were seeded in a low-attachment dish, and exhibited constant growth and invasion. Additionally, we demonstrated the feasibility of high-throughput drug screening using these cells. In conclusion, the NCC-LGFMS1-C1 cell line is a useful tool for studying LGFMS.
低度恶性纤维黏液样肉瘤（LGFMS）是一种罕见的软组织肉瘤，其遗传特征为存在 FUS-CREB3L2 基因融合。尽管 LGFMS 在早期阶段表现出惰性特征，但该疾病的复发率、转移率和死亡率都很高。目前，FUS-CREB3L2 基因融合在 LGFMS 独特特征中的作用尚不清楚，也没有改善 LGFMS 患者临床结局的方法；因此，需要对 LGFMS 进行广泛的研究。患者来源的癌细胞系是癌症研究的重要工具，但尚未为 LGFMS 建立细胞系。在这里，我们旨在开发用于 LGFMS 的新型细胞系，并成功地使用手术切除的肿瘤组织建立了该细胞系。这些细胞被命名为 NCC-LGFMS1-C1，它们具有与原始肿瘤相同的融合基因和可见的拷贝数变异。这些细胞呈成纤维细胞样外观，在低附着培养皿中接种时形成球体，并表现出持续的生长和侵袭。此外，我们证明了使用这些细胞进行高通量药物筛选的可行性。总之，NCC-LGFMS1-C1 细胞系是研究 LGFMS 的有用工具。
