Sorrentino Stefania, Ash Shifra, Haupt Riccardo, Plantaz Dominique, Schiff Isabelle, Hero Barbara, Simon Thorsten, Kachanov Denis, Shamanskaya Tatyana, Kraal Katheljine, Littooij Annemieke, Wieczoreck Alexsandra, Balwierz Walentyna, Laureys Geneviève, Trager Catherine, Sertorio Fiammetta, Erminio Giovanni, Fragola Martina, Beck Popovic Maja, De Bernardi Bruno, Trahair Toby
Paediatric Oncology Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Joan and Sanford Weill Paediatric Haematology Oncology and Bone Marrow Transplantation Division, Ruth and Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel.
Front Pediatr. 2022 Oct 10;10:1023498. doi: 10.3389/fped.2022.1023498. eCollection 2022.
Between 5 and 15% of children with neuroblastoma (NB) present with or develop spinal canal invasion (SCI). The majority of these children have symptoms of epidural compression of spinal cord and/or spinal nerves. Treatment of NB-SCI is considered an emergency but its modalities are not yet well-established. Independently of treatment, NB-SCI may result in significant long-term disabilities. We report on the first prospective study of NB-SCI focused on presenting characteristics of both symptomatic and asymptomatic patients and correlation between SCI-related symptoms and imaging features.
This SIOPEN prospective NB-SCI study opened in June 2014. Patient data including SCI symptoms evaluated by standardized measures and spinal cord imaging studies were collected for each patient. For the purpose of this study data entry was locked on July 2021.
Of the 208 NB-SCI patients registered, 196 were evaluable for this analysis of whom 67% were symptomatic and 33% asymptomatic. Median age was 11 months. The thorax was the commonest primary tumor site. The median intervals between initial symptoms and diagnosis and between first medical visit and diagnosis were 14 and 3 days, respectively. The was no statistical difference in frequency of presenting characteristics between symptomatic and asymptomatic patients. Presenting features of NB-SCI patients differed from other NBs for older median age, prevalence of thoracic vs. abdominal primary site, prevalence of localized vs. metastatic disease and lower incidence of MYCN gene amplification. The most common SCI features were motor deficit in the younger and pain in the older patients that correlated on imaging with both transverse and longitudinal extent but not with the level of intraspinal tumor. Spinal cord T2-hyperintensity was more frequently detected in symptomatic patients (not significant).
This prospective study confirms that children with NB-SCI differ from NBs without SCI. Compared to previous studies, it provides more detailed information regarding presenting symptoms, time intervals between SCI symptoms, medical visit and diagnosis, and correlations between symptoms and imaging features.
5%至15%的神经母细胞瘤（NB）患儿会出现或发展为椎管侵犯（SCI）。这些患儿中的大多数有脊髓和/或脊神经硬膜外压迫症状。NB-SCI的治疗被视为紧急情况，但其治疗方式尚未完全确立。无论治疗如何，NB-SCI都可能导致严重的长期残疾。我们报告了第一项关于NB-SCI的前瞻性研究，重点关注有症状和无症状患者的表现特征以及SCI相关症状与影像学特征之间的相关性。
这项SIOPEN前瞻性NB-SCI研究于2014年6月启动。收集了每位患者的包括通过标准化测量评估的SCI症状和脊髓影像学研究在内的患者数据。出于本研究的目的，数据录入于2021年7月锁定。
在登记的208例NB-SCI患者中，196例可用于本分析，其中67%有症状，33%无症状。中位年龄为11个月。胸部是最常见的原发肿瘤部位。初始症状与诊断之间以及首次就诊与诊断之间的中位间隔分别为14天和3天。有症状和无症状患者的表现特征频率无统计学差异。NB-SCI患者的表现特征与其他NB患者不同，在于中位年龄较大、胸部与腹部原发部位的患病率、局限性与转移性疾病的患病率以及MYCN基因扩增的发生率较低。最常见的SCI特征是年轻患者的运动功能障碍和年长患者的疼痛，在影像学上与横向和纵向范围相关，但与椎管内肿瘤的水平无关。脊髓T2高信号在有症状患者中更常被检测到（无显著性差异）。
这项前瞻性研究证实，NB-SCI患儿与无SCI的NB患儿不同。与以往研究相比，它提供了关于表现症状、SCI症状、就诊与诊断之间的时间间隔以及症状与影像学特征之间相关性的更详细信息。
