Zhao Manli, Gu Weizhong, Liu Fei, Yu Lihua, Shu Yan, Liu Lei, Hu Jiahui, Liu Yang, Tang Hongfeng, Mao Jianhua
Department of Pathology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China.
Department of Nephrology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, China.
Pediatr Dev Pathol. 2023 Mar-Apr;26(2):124-132. doi: 10.1177/10935266231151316. Epub 2023 Feb 12.
gene amplification is a powerful indicator of poor prognosis of neuroblastoma patients. However, non-amplified patients still showed heterogeneity in survival outcome. This study aimed to investigate the prognostic role of MYCN immunohistochemistry (IHC) in pre-treatment and post-treatment neuroblastoma tumors.
215 untreated neuroblastoma tumors were stained with anti-MYCN antibody by immunohistochemical staining. 22 post-treatment tumors were used to compare MYCN staining with paired pre-treatment samples. Results were analyzed with other prognostic indicators.
Moderate or strong expression of MYCN was associated with unfavorable survival outcomes ( < .001). Prominent staining of MYCN IHC was 95% sensitive and 95% specific for the presence of gene amplification in this study. Ten of 214 (5%) patients showed prominent MYCN staining but non-amplification, and had a poor prognosis (29.6 ± 16.4%, 5-year overall survival). Most of cases (7/11, 64%) with high or moderate MYCN expression before chemotherapy showed lower expression in their tumors after chemotherapy.
MYCN protein overexpression was not only a sensitive and specific marker for gene amplification, but also a marker of poor prognosis in patients without amplification. However, MYCN protein expression was not always consistent before and after treatment.
基因扩增是神经母细胞瘤患者预后不良的有力指标。然而，未扩增的患者在生存结局上仍表现出异质性。本研究旨在探讨MYCN免疫组织化学（IHC）在治疗前和治疗后神经母细胞瘤肿瘤中的预后作用。
采用免疫组织化学染色法，用抗MYCN抗体对215例未经治疗的神经母细胞瘤肿瘤进行染色。使用22例治疗后的肿瘤样本与配对的治疗前样本比较MYCN染色情况。将结果与其他预后指标进行分析。
MYCN的中度或强表达与不良生存结局相关（P<0.001）。在本研究中，MYCN免疫组化的显著染色对基因扩增的存在具有95%的敏感性和95%的特异性。214例患者中有10例（5%）表现出MYCN显著染色但未扩增，预后较差（5年总生存率为29.6±16.4%）。大多数化疗前MYCN表达高或中度的病例（7/11，64%）在化疗后的肿瘤中表达降低。
MYCN蛋白过表达不仅是基因扩增的敏感和特异性标志物，也是未扩增患者预后不良的标志物。然而，MYCN蛋白表达在治疗前后并不总是一致的。
