Alveolar rhabdomyosarcoma (aRMS) is a histological subtype of RMS, which is the most common pediatric and adolescent soft-tissue sarcoma, accounting for 3–4% of all pediatric malignancies. Patient-derived cells are essential tools for understanding the molecular mechanisms of poor prognosis and developing novel anti-cancer drugs. However, only a limited number of well-characterized cell lines for rhabdomyosarcoma from public cell banks is available. Therefore, we aimed to establish a novel cell line of aRMS from the tumor tissue of a patient with aRMS. The cell line was established from surgically resected tumor tissue from a 4-year-old male patient diagnosed with stage III, T2bN1M0 aRMS and was named as NCC-aRMS1-C1. The cells were maintained for more than 3 months under tissue culture conditions and passaged more than 20 times. We confirmed the presence of identical fusion gene such as PAX7-FOXO1 in both the original tumor and NCC-aRMS1-C1. The cells exhibited spheroid formation and invasion. We found that docetaxel, vincristine, ifosfamide, dacarbazine, and romidepsin showed remarkable growth-suppressive effects on the NCC-aRMS1-C1 cells. In conclusion, the NCC-aRMS1-C1 cell line exhibited characteristics that may correspond to the lymph node metastasis in aRMS and mirror its less aggressive features. Thus, it may be useful for innovative seeds for novel therapeutic strategies.

肺泡横纹肌肉瘤（aRMS）是RMS的一种组织学亚型，是最常见的小儿和青少年软组织肉瘤，占所有小儿恶性肿瘤的3-4％。患者来源的细胞是了解不良预后的分子机制和开发新型抗癌药物的重要工具。然而，仅有限数量的来自公共细胞库的横纹肌肉瘤的良好表征的细胞系可用。因此，我们旨在从患有aRMS的患者的肿瘤组织中建立新型的aRMS细胞系。该细胞系是从一名手术切除的肿瘤组织中建立的，该组织来自一名诊断为III期T2bN1M0 aRMS的4岁男性患者，并命名为NCC-aRMS1-C1。在组织培养条件下将细胞维持3个月以上，并传代20次以上。原发性肿瘤和NCC-aRMS1-C1中均存在PAX7-FOXO1。细胞表现出球状的形成和侵袭。我们发现多西他赛，长春新碱，异环磷酰胺，达卡巴嗪和罗米地辛对NCC-aRMS1-C1细胞显示出显着的生长抑制作用。总之，NCC-aRMS1-C1细胞系表现出可能与aRMS淋巴结转移相对应的特征，并反映了其侵略性较低的特征。因此，对于用于新颖治疗策略的创新种子可能是有用的。