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

Stefania Sorrentino; Shifra Ash; Riccardo Haupt; Dominique Plantaz; Isabelle Schiff; Barbara Hero; Thorsten Simon; Denis Kachanov; Tatyana Shamanskaya; Katheljine Kraal; Annemieke Littooij; Alexsandra Wieczoreck; Walentyna Balwierz; Geneviève Laureys; Catherine Trager; Fiammetta Sertorio; Giovanni Erminio; Martina Fragola; Maja Beck Popovic; Bruno De Bernardi; Toby Trahair

doi:10.3389/fped.2022.1023498

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

Front Pediatr. 2022 Oct 10:10:1023498. doi: 10.3389/fped.2022.1023498. eCollection 2022.

Authors

Stefania Sorrentino¹, Shifra Ash², Riccardo Haupt³, Dominique Plantaz⁴, Isabelle Schiff⁴, Barbara Hero⁵, Thorsten Simon⁵, Denis Kachanov⁶, Tatyana Shamanskaya⁶, Katheljine Kraal⁷, Annemieke Littooij⁷, Alexsandra Wieczoreck⁸, Walentyna Balwierz⁸, Geneviève Laureys⁹, Catherine Trager¹⁰, Fiammetta Sertorio¹¹, Giovanni Erminio¹², Martina Fragola¹², Maja Beck Popovic¹³, Bruno De Bernardi¹, Toby Trahair^{14

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Affiliations

¹ Paediatric Oncology Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
² 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.
³ DOPO Clinic, IRCCS Istituto Giannina Gaslini, Genova, Italy.
⁴ Department de Paediatrics, Hôpital Couple Enfants, CHU Grenoble, Grenoble, France.
⁵ Department of Paediatric Haematology and Oncology, Children's Hospital, University of Cologne, Cologne, Germany.
⁶ Department of Clinical Oncology, Dmitry Rogachev National Medical Research Center of Paediatric Haematology, Oncology and Immunology, Moscow, Russia.
⁷ Princess Màxima Centre for Paediatric Oncology, Utrecht, Netherlands.
⁸ Paediatric Oncology and Haematology Department, Institute of Paediatrics, Jagiellonian University, Krakow, Poland.
⁹ Department of Paediatric Haematology-Oncology, Prinses Elisabeth Kinderziekenhuis, University Hospital, Gent, Belgium.
¹⁰ Women's and Childrens Health, Karolinska Institutet, Stockholm, Sweden.
¹¹ Radiology Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
¹² Scientific Directorate, IRCCS Istituto Giannina Gaslini, Genova, Italy.
¹³ Centre Hospitalier Universitaire Vaudois, Unité d'Hémato-Oncologie Pédiatrique, Lausanne, Switzerland.
¹⁴ Kids Cancer Center, Sydney Children's Hospital, Randwick, NSW, Australia.
¹⁵ Children's Cancer Institute, Lowy Cancer Research Centre, University of New South Wales, Kensington, NSW, Australia.
¹⁶ School of Women's and Children's Health, University of New South Wales, Kensington, NSW, Australia.

Abstract

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.

Keywords: epidural spinal cord compression; long-term disabilities; neuroblastoma; pediatric tumors; spinal canal invasion.

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.