Iwata Shuhei, Noguchi Rei, Osaki Julia, Adachi Yuki, Shiota Yomogi, Osaki Shuhei, Nishino Shogo, Yoshida Akihiko, Ohtori Seiji, Kawai Akira, Kondo Tadashi
Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Department of Orthopaedic Surgery, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan.
Hum Cell. 2025 Jun 28;38(4):122. doi: 10.1007/s13577-025-01250-7.
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma characterized by a myxoid matrix and a distinctive lobulated architecture, composed of cords and clusters of uniform round-to-rhabdoid cells. At the molecular level, EMC is defined by specific gene fusions involving NR4A3, most frequently EWSR1::NR4A3. The responses to conventional chemotherapy are limited, and the prognosis for patients with advanced or metastatic disease remains poor. We successfully developed the NCC-EMC1-C1 cell line using surgically resected tumor tissue from a patient with EMC. NCC-EMC1-C1 cells exhibited constant proliferation in monolayer culture, spheroid formation in low-attachment plates, and migration. High-throughput screening of 221 anticancer drugs using NCC-EMC1-C1 identified three candidates, brigatinib, panobinostat, and romidepsin, that demonstrated low IC values. These data indicated the utility of NCC-EMC1-C1 for the experiments based on screening. We conclude that NCC-EMC1-C1 is a valuable tool for preclinical and basic research on EMC.
骨外黏液样软骨肉瘤（EMC）是一种罕见的软组织肉瘤，其特征为黏液样基质和独特的分叶状结构，由条索状和簇状的形态一致的圆形至横纹肌样细胞组成。在分子水平上，EMC由涉及NR4A3的特定基因融合所定义，最常见的是EWSR1::NR4A3。对传统化疗的反应有限，晚期或转移性疾病患者的预后仍然很差。我们使用一名EMC患者手术切除的肿瘤组织成功建立了NCC-EMC1-C1细胞系。NCC-EMC1-C1细胞在单层培养中表现出持续增殖，在低附着板中形成球体，并具有迁移能力。使用NCC-EMC1-C1对221种抗癌药物进行高通量筛选，确定了三种候选药物，即布加替尼、帕比司他和罗米地辛，它们显示出较低的半数抑制浓度（IC）值。这些数据表明NCC-EMC1-C1在基于筛选的实验中的实用性。我们得出结论，NCC-EMC1-C1是EMC临床前和基础研究的宝贵工具。
