Wang Haoru, Li Ting, Ni Xiaoying, Chen Xin, He Ling, Cai Jinhua
Department of Radiology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders, No. 136 Zhongshan Road 2, Yuzhong District, Chongqing, 400014, China.
Abdom Radiol (NY). 2024 Jun;49(6):1949-1960. doi: 10.1007/s00261-024-04196-w. Epub 2024 Mar 4.
The MYCN oncogene is a critical factor in the development and progression of neuroblastoma, and image-defined risk factors (IDRFs) are radiological findings used for the preoperative staging of neuroblastoma. This study aimed to investigate the specific categories of IDRFs associated with MYCN amplification in neuroblastoma and their association with overall survival.
A retrospective analysis was conducted on a cohort of 280 pediatric patients diagnosed with neuroblastoma, utilizing a combination of clinical and radiological data. MYCN amplification status was ascertained through molecular testing, and the assessment of IDRFs was conducted using either contrast-enhanced computed tomography or magnetic resonance imaging. The specific categories of IDRFs associated with MYCN amplification and their association with overall survival were analyzed.
MYCN amplification was identified in 19.6% (55/280) of patients, with the majority of primary lesions located in the abdomen (53/55, 96.4%). Lesions accompanied by MYCN amplification exhibited significantly larger tumor volume and a greater number of IDRFs compared with those without MYCN amplification (P < 0.001). Both univariate and multivariate analyses revealed that coeliac axis/superior mesenteric artery encasement and infiltration of adjacent organs/structures were independently associated with MYCN amplification in abdominal neuroblastoma (P < 0.05). Patients presenting with more than four IDRFs experienced a worse prognosis (P = 0.017), and infiltration of adjacent organs/structures independently correlated with overall survival in abdominal neuroblastoma (P = 0.009).
The IDRFs are closely correlated with the MYCN amplification status and overall survival in neuroblastoma.
MYCN癌基因是神经母细胞瘤发生发展的关键因素，图像定义风险因素（IDRFs）是用于神经母细胞瘤术前分期的影像学表现。本研究旨在探讨神经母细胞瘤中与MYCN扩增相关的IDRFs的具体类别及其与总生存期的关系。
对280例诊断为神经母细胞瘤的儿科患者队列进行回顾性分析，综合利用临床和影像学数据。通过分子检测确定MYCN扩增状态，使用对比增强计算机断层扫描或磁共振成像对IDRFs进行评估。分析与MYCN扩增相关的IDRFs的具体类别及其与总生存期的关系。
19.6%（55/280）的患者检测到MYCN扩增，大多数原发灶位于腹部（53/55，96.4%）。与未发生MYCN扩增的病灶相比，伴有MYCN扩增的病灶肿瘤体积明显更大，IDRFs数量更多（P < 0.001）。单因素和多因素分析均显示，腹腔干/肠系膜上动脉包绕以及邻近器官/结构浸润与腹部神经母细胞瘤的MYCN扩增独立相关（P < 0.05）。出现超过4种IDRFs的患者预后较差（P = 0.017），邻近器官/结构浸润与腹部神经母细胞瘤的总生存期独立相关（P = 0.009）。
IDRFs与神经母细胞瘤的MYCN扩增状态和总生存期密切相关。
