Universität zu Köln

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, Genevieve, Trager, Catherine, Sertorio, Fiammetta, Erminio, Giovanni, Fragola, Martina, Beck Popovic, Maja, De Bernardi, Bruno and Trahair, Toby (2022). Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe-Neuroblastoma (SIOPEN). Front. Pediatr., 10. LAUSANNE: FRONTIERS MEDIA SA. ISSN 2296-2360

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Abstract

IntroductionBetween 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. Materials and methodsThis 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. ResultsOf 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). ConclusionThis 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Sorrentino, Stefania UNSPECIFIED UNSPECIFIED UNSPECIFIED Ash, Shifra UNSPECIFIED UNSPECIFIED UNSPECIFIED Haupt, Riccardo UNSPECIFIED UNSPECIFIED UNSPECIFIED Plantaz, Dominique UNSPECIFIED UNSPECIFIED UNSPECIFIED Schiff, Isabelle UNSPECIFIED UNSPECIFIED UNSPECIFIED Hero, Barbara UNSPECIFIED UNSPECIFIED UNSPECIFIED Simon, Thorsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Kachanov, Denis UNSPECIFIED UNSPECIFIED UNSPECIFIED Shamanskaya, Tatyana UNSPECIFIED UNSPECIFIED UNSPECIFIED Kraal, Katheljine UNSPECIFIED UNSPECIFIED UNSPECIFIED Littooij, Annemieke UNSPECIFIED UNSPECIFIED UNSPECIFIED Wieczoreck, Alexsandra UNSPECIFIED UNSPECIFIED UNSPECIFIED Balwierz, Walentyna UNSPECIFIED UNSPECIFIED UNSPECIFIED Laureys, Genevieve UNSPECIFIED UNSPECIFIED UNSPECIFIED Trager, Catherine UNSPECIFIED UNSPECIFIED UNSPECIFIED Sertorio, Fiammetta UNSPECIFIED UNSPECIFIED UNSPECIFIED Erminio, Giovanni UNSPECIFIED UNSPECIFIED UNSPECIFIED Fragola, Martina UNSPECIFIED UNSPECIFIED UNSPECIFIED Beck Popovic, Maja UNSPECIFIED UNSPECIFIED UNSPECIFIED De Bernardi, Bruno UNSPECIFIED UNSPECIFIED UNSPECIFIED Trahair, Toby UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-694392
DOI:	10.3389/fped.2022.1023498
Journal or Publication Title:	Front. Pediatr.
Volume:	10
Date:	2022
Publisher:	FRONTIERS MEDIA SA
Place of Publication:	LAUSANNE
ISSN:	2296-2360
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SYMPTOMATIC EPIDURAL COMPRESSION; CORD COMPRESSION; INTRASPINAL EXTENSION; RISK GROUP; CLASSIFICATION; CHEMOTHERAPY; DIAGNOSIS; CHILDREN Multiple languages Pediatrics Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69439