Retinoblastoma (RB) is a childhood eye tumor, caused by the RB1 gene mutation. Since RB is a rapidly proliferating tumor, the patient presents with a Group-D/E tumor at the time of diagnosis. Enucleation is preferred in most unilateral cases to prevent metastasis. Various cell lines have been established to study the tumor's growth pattern and target the cancer cells. The commonly used cell lines are WERI-Rb-1 and Y79, both isolated from the primary tumor of RB. Cell lines established from the metastatic site of RB have not been characterized before. In this study, we have characterized NCC-RbC-51, derived from RB tumor to cervical lymph node site and investigated its potential to represent a highly aggressive and metastatic tumor. We compared the proliferative and invasive properties of NCC-RbC-51 with a cell line isolated from the primary site, WERI-Rb-1. NCC-RbC-51 had higher rates of proliferation and apoptosis and had better invasive ability. Copy number variation analysis and the pathways predicted from these show that the pathways altered in NCC-RbC-51 could contribute to its metastatic nature. In all, the results suggest that NCC-RbC-51, a cell line isolated from metastatic site, could be a potential model to study aggressive/invasive RB.

中文翻译：

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NCC-RbC-51，一种从转移部位分离的RB细胞系的特征。

视网膜母细胞瘤（RB）是由RB1基因突变引起的儿童眼部肿瘤。由于RB是一种快速增殖的肿瘤，因此患者在诊断时会出现D / E组肿瘤。在大多数单侧病例中，最好采用去核术以防止转移。已经建立了各种细胞系来研究肿瘤的生长方式并靶向癌细胞。常用的细胞系是WERI-Rb-1和Y79，两者均从RB的原发肿瘤中分离出来。从RB的转移位点建立的细胞系以前没有被鉴定过。在这项研究中，我们表征了NCC-RbC-51，其从RB肿瘤衍生至宫颈淋巴结部位，并研究了其代表高度侵袭性和转移性肿瘤的潜力。我们将NCC-RbC-51与从主要部位WERI-Rb-1分离的细胞系的增殖和侵袭特性进行了比较。NCC-RbC-51具有较高的增殖和凋亡率，并具有更好的侵袭能力。拷贝数变异分析和由此预测的途径表明，NCC-RbC-51中改变的途径可能有助于其转移性质。总之，结果表明，从转移部位分离的细胞系NCC-RbC-51可能是研究侵袭性/侵袭性RB的潜在模型。