Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

Anna Gabryś; Julia Kuzaj; Dominika Pawełczak; Katarzyna Seliga; Agnieszka Jelińska; Andrzej Tysarowski; Wiesława Grajkowska; Stanisław Kwiatkowski

doi:10.1055/s-0040-1716547

Journal of Pediatric Neurology, Table of Contents

Journal of Pediatric Neurology 2021; 19(04): 259-263
DOI: 10.1055/s-0040-1716547

Case Report

Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

Authors

Anna Gabryś

¹Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
Julia Kuzaj

¹Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
Dominika Pawełczak

¹Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
Katarzyna Seliga

²Molecular and Translational Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
Agnieszka Jelińska

³Jagiellonian University Medical College, Kraków, Poland
Andrzej Tysarowski

⁴Department of Pathology and Laboratory Medicine, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

²Molecular and Translational Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
Wiesława Grajkowska

⁵Department of Pathology, Children's Memorial Health Institute, Warsaw, Poland
Stanisław Kwiatkowski

⁶Jagiellonian University Medical College, Clinic of Children's Neurosurgery, Kraków, Poland

Abstract

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is an entity introduced in 2016 World Health Organization classification of tumors of the central nervous system. The tumor occurs very rarely. Due to the lack of specific clinical and radiological features, biopsy is necessary to be performed and histological and immunohistochemical testing is essential to reach the diagnosis. A 4.5-year-old girl presented with a history of headache, vomiting, and right eye convergent squint. Imaging revealed multiple enhancing lesions located supra- and infratentorially and intramedullary. Histopathological examination demonstrated diffused growth of neoplastic cells. Molecular testing revealed KIAA1549-BRAF fusion and the diagnosis of DLGNT was stated.

Keywords

diffuse leptomeningeal glioneuronal tumor - leptomeningeal enhancement - pediatric oncology

Full Text

References

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