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Lookup NU author(s): Professor Simon BaileyORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© The Author(s) 2024.Purpose: Brain stem tumors in children < 3 months at diagnosis are extremely rare. Our aim is to study a retrospective cohort to improve the understanding of the disease course and guide patient management. Methods: This is a multicenter retrospective analysis across the European Society for Pediatric Oncology SIOP-E HGG/DIPG Working Group linked centers, including patients with a brainstem tumor diagnosed between 2009 and 2020 and aged < 3 months at diagnosis. Clinical data were collected, and imaging characteristics were analyzed blindly and independently by two neuroradiologists. Results: Five cases were identified. No patient received any therapy. The epicenter of two tumors was in the medulla oblongata alone and in the medulla oblongata and the pons in three. For patients with tumor in equal parts in the medulla oblongata and the pons (n = 3), the extension at diagnosis involved the spinal cord; for the two patients with the tumor epicenter in the medulla oblongata alone (n = 2), the extension at diagnosis included the pons (n = 2) and the spinal cord (n = 1). Biopsy was performed in one patient identifying a pilocytic astrocytoma. Two patients died. In one patient, autopsy revealed a high-grade glioma (case 3). Three survivors showed either spontaneous tumor regression (n = 2) or stable disease (n = 1). Survivors were followed up for 10, 7, and 0.6 years, respectively. One case had the typical imaging characteristics of a dorsal exophytic low-grade glioma. Conclusions: No patient fulfilled the radiologic criteria defining a high-grade glioma. Central neuroradiological review and biopsy may provide useful information regarding the patient management.
Author(s): Papangelopoulou D, Bison B, Behrens L, Bailey S, Ansari M, Ehlert K, Martinez OC, Kramm CM, Morales La Madrid A, von Bueren AO
Publication type: Article
Publication status: Published
Journal: Child's Nervous System
Year: 2024
Volume: 40
Pages: 1053-1064
Online publication date: 20/02/2024
Acceptance date: 26/12/2023
Date deposited: 20/03/2024
ISSN (print): 0256-7040
ISSN (electronic): 1433-0350
Publisher: Springer Science and Business Media Deutschland GmbH
URL: https://doi.org/10.1007/s00381-023-06272-w
DOI: 10.1007/s00381-023-06272-w
Data Access Statement: The online version contains supplementary material available at https://doi. org/10. 1007/ s00381- 023- 06272-w.
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