■高风险和复发性神经母细胞瘤(NB)患者的预后仍然很差。肿瘤相关标志物的鉴定对于鉴别诊断很重要,预后,以及靶向治疗的发展。该研究的目的是确定9种最常见的NB抗原的表达谱,并评估其与临床病理特征和患者生存率的关系。
■使用定量PCR评估来自86例NB患者的肿瘤样品中肿瘤相关抗原(TAA)的表达。21名良性肿瘤患者和17名健康供体被指定为对照。
■酪氨酸羟化酶(TH)的过表达,PHOX2B,PRAME,GPC2、B7-H3和Survivin是最典型的NB。检测到MAGEA3、MAGEA1和NY-ESO-1在低水平的阳性表达占54%,48%,52%,分别,并且不是NB特异性的。在没有MYCN扩增的样品中观察到更高的TH表达,而Survivin的表达较高,PHOX2B,GPC2与1p.36缺失的存在显著相关。TH的过表达,PHOX2B,MAGEA1与更好的无事件生存(EFS)和总生存(OS)相关.Survivin过度表达与不良EFS相关,但对OS没有影响。多因素分析证实Survivin是低生存率的独立标志物。和PHOX2B和MAGEA1更好的生存。
■TH的高表达,PHOX2B,MAGEA1基因是OS和EFS的有利预后因素,而Survivin的高表达与复发或进展的风险增加相关。
UNASSIGNED: The prognosis of high-risk and relapsed neuroblastoma (NB) patients remains poor. The identification of tumor-associated markers is important for differential diagnosis, prognosis, and the development of targeted therapies. The aim of the study was to determine the expression profile of nine most common NB antigens and assess their association with clinicopathological characteristics and patient survival.
UNASSIGNED: Tumor samples from 86 patients with NB were evaluated for the expression of tumor-associated antigen (TAA) using quantitative PCR. Twenty-one patients with benign tumors and 17 healthy donors were assigned as controls.
UNASSIGNED: Overexpression of tyrosine hydroxylase (TH),
PHOX2B, PRAME, GPC2, B7-H3, and Survivin is the most typical for NB. Positive expression of MAGEA3, MAGEA1, and NY-ESO-1 at low levels was detected in 54%, 48%, and 52%, respectively, and was not NB specific. Higher TH expression was observed in samples without MYCN-amplification, while higher expression of Survivin,
PHOX2B, and GPC2 was significantly associated with the presence of 1p.36 deletion. Overexpression of TH,
PHOX2B, and MAGEA1 was associated with better event-free (EFS) and overall survival (OS). Survivin overexpression was associated with poor EFS but had no impact on OS. Multivariate analysis confirmed Survivin as independent marker for poor survival, and
PHOX2B and MAGEA1 for better survival.
UNASSIGNED: High expression of TH,
PHOX2B, and MAGEA1 genes are favorable prognostic factors for OS and EFS, whereas high expression of Survivin is associated with an increased risk of relapse or progression.