Brain stem tumors in children less than 3 months: Clinical and radiologic findings of a rare disease
Por:
Papangelopoulou D, Bison B, Behrens L, Bailey S, Ansari M, Ehlert K, Cruz-Martínez O, Kramm CM, Morales La Madrid A and von Bueren AO
Publicada:
20 feb 2024
Ahead of Print:
1 feb 2024
Resumen:
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
Filiaciones:
Papangelopoulou D:
Department of Pediatrics, Gynecology and Obstetrics, Division of General Pediatrics, Pediatric Hematology and Oncology Unit, University Hospitals of Geneva, Geneva, Switzerland
Cansearch Research Platform for Pediatric Oncology and Hematology, Faculty of Medicine, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
Bison B:
Diagnostic and Interventional Neuroradiology, Faculty of Medicine, University of Augsburg, Augsburg, Germany
Behrens L:
Diagnostic and Interventional Neuroradiology, Faculty of Medicine, University of Augsburg, Augsburg, Germany
Bailey S:
Wolfson Childhood Cancer Research Centre, Newcastle University Centre for Cancer, Newcastle upon Tyne, UK
Ansari M:
Department of Pediatrics, Gynecology and Obstetrics, Division of General Pediatrics, Pediatric Hematology and Oncology Unit, University Hospitals of Geneva, Geneva, Switzerland
Cansearch Research Platform for Pediatric Oncology and Hematology, Faculty of Medicine, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
Ehlert K:
Department of Pediatric Hematology and Oncology, University Medicine Greifswald, Greifswald, Germany
Cruz-Martínez O:
Pediatric Cancer Center Barcelona, Hospital Sant Joan de Déu, Barcelona, Spain
Kramm CM:
Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University Medical Center Goettingen, Goettingen, Germany
Morales La Madrid A:
Pediatric Cancer Center Barcelona, Hospital Sant Joan de Déu, Barcelona, Spain
von Bueren AO:
Department of Pediatrics, Gynecology and Obstetrics, Division of General Pediatrics, Pediatric Hematology and Oncology Unit, University Hospitals of Geneva, Geneva, Switzerland. Andre.vonBuren@hcuge.ch
Cansearch Research Platform for Pediatric Oncology and Hematology, Faculty of Medicine, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland. Andre.vonBuren@hcuge.ch
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