Myxoid liposarcoma is a rare mesenchymal malignancy, which is characterized by a FUS-DDIT3 fusion known as chromosomal translocation t(12;16)(q13;p11) and arises in the fat tissue. Although surgery with radiation has been established as a standard treatment, myxoid liposarcoma shows frequent recurrence and poor prognosis, thus requiring new therapeutic approaches. Patient-derived cell lines represent a critical tool for basic and preclinical research. However, only two such myxoid liposarcoma cell lines have been reported, and they are not available in cell banks. The aim of this study was to establish and characterize a novel myxoid liposarcoma cell line. Using surgically resected tumor tissue from a 47-year-old male patient, we established the NCC-MLPS1-C1 cell line. NCC-MLPS1-C1 cells were characterized by FUS-DDIT3 fusion, slow growth, spheroid formation, and invasive capability. We screened the effect of anti-cancer agents on the proliferation of NCC-MLPS1-C1 cells. The cells displayed a remarkable response to multitarget kinase inhibitors of RET, PDGFR-β, VEGFR, or FGFR. NCC-MLPS1-C1 cells and the tumor tissue shared common profiles of kinase activity including identified drug targets, such as RET and PDGFR-β. We believe that NCC-MLPS1-C1 cells will represent a useful tool for basic and preclinical studies of myxoid liposarcoma.

粘液样脂肪肉瘤是一种罕见的间充质恶性肿瘤，其特征是FUS-DDIT3融合，称为染色体易位 t(12;16)(q13;p11)，起源于脂肪组织。尽管放射手术已成为标准治疗方法，但粘液样脂肪肉瘤复发频繁且预后不良，因此需要新的治疗方法。源自患者的细胞系是基础和临床前研究的关键工具。然而，只有两种这样的粘液样脂肪肉瘤细胞系被报道，并且它们在细胞库中不可用。本研究的目的是建立和表征一种新的粘液样脂肪肉瘤细胞系。使用从一名 47 岁男性患者手术切除的肿瘤组织，我们建立了 NCC-MLPS1-C1 细胞系。NCC-MLPS1-C1 细胞的特点是FUS-DDIT3融合、生长缓慢、球体形成和侵袭能力。我们筛选了抗癌剂对 NCC-MLPS1-C1 细胞增殖的影响。这些细胞对 RET、PDGFR-β、VEGFR 或 FGFR 的多靶点激酶抑制剂表现出显着的反应。NCC-MLPS1-C1 细胞和肿瘤组织共享激酶活性的共同特征，包括确定的药物靶点，如 RET 和 PDGFR-β。我们相信 NCC-MLPS1-C1 细胞将成为粘液样脂肪肉瘤基础和临床前研究的有用工具。