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Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)

INTRODUCTION: 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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9589152/
https://www.ncbi.nlm.nih.gov/pubmed/36299690
http://dx.doi.org/10.3389/fped.2022.1023498
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author 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
author_facet 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
author_sort Sorrentino, Stefania
collection PubMed
description INTRODUCTION: 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. MATERIALS AND METHODS: 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. RESULTS: 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). CONCLUSION: 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.
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spelling pubmed-95891522022-10-25 Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN) 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 Front Pediatr Pediatrics INTRODUCTION: 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. MATERIALS AND METHODS: 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. RESULTS: 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). CONCLUSION: 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. Frontiers Media S.A. 2022-10-10 /pmc/articles/PMC9589152/ /pubmed/36299690 http://dx.doi.org/10.3389/fped.2022.1023498 Text en Copyright © 2022 Sorrentino, Ash, Haupt, Plantaz, Schiff, Hero, Simon, Kachanov, Shamanskaya, Kraal, Littooij, Wieczoreck, Balwierz, Laureys, Trager, Sertorio, Erminio, Fragola, Beck Popovic, De Bernardi and Trahair. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
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
Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title_full Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title_fullStr Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title_full_unstemmed Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title_short Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN)
title_sort presenting features of neuroblastoma with spinal canal invasion. a prospective study of the international society of pediatric oncology europe - neuroblastoma (siopen)
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9589152/
https://www.ncbi.nlm.nih.gov/pubmed/36299690
http://dx.doi.org/10.3389/fped.2022.1023498
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