Journal of Pediatric Neurology
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

Anna Gabryś
1  Jagiellonian University Medical College, Faculty of Medicine, Kraków, Poland
,
Julia Kuzaj
1  Jagiellonian University Medical College, Faculty of Medicine, Kraków, Poland
,
Dominika Pawełczak
1  Jagiellonian University Medical College, Faculty of Medicine, Kraków, Poland
,
Katarzyna Seliga
2  Molecular and Translational Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
,
Agnieszka Jelińska
3  Jagiellonian University Medical College, Kraków, Poland
,
Andrzej Tysarowski
4  Department of Pathology and Laboratory Medicine, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
2  Molecular and Translational Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
,
Wiesława Grajkowska
5  Department of Pathology, Children's Memorial Health Institute, Warsaw, Poland
,
Stanisław Kwiatkowski
6  Jagiellonian University Medical College, Clinic of Children's Neurosurgery, Kraków, Poland
› Author Affiliations

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.



Publication History

Received: 08 July 2020

Accepted: 09 August 2020

Publication Date:
14 September 2020 (online)

Georg Thieme Verlag KG
Stuttgart · New York