Download PDF
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

Anna Gabryś
1   Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
,
Julia Kuzaj
1   Faculty of Medicine, Jagiellonian University Medical College, Kraków, Poland
,
Dominika Pawełczak
1   Faculty of Medicine, Jagiellonian University Medical College, 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
› Further Information
Also available at
    Permissions and Reprints

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


Publication History

Received: 08 July 2020

Accepted: 09 August 2020

Article published online:
14 September 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Wesseling P, Capper D. WHO 2016 classification of gliomas. Neuropathol Appl Neurobiol 2018; 44 (02) 139-150
    CrossrefPubMedSearch in Google Scholar
  • 2 Gardiman MP, Fassan M, Orvieto E. et al. Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain pathology ISSN 2008: 1015-6305
  • 3 Fiaschi P, Badaloni F, Cagetti B. et al. Disseminated oligodendroglial-like leptomeningeal tumor in the adult: case report and review of the literature. World Neurosurg 2018; 114: 53-57
    CrossrefPubMedSearch in Google Scholar
  • 4 Chen W, Kong Z, Fu J. et al. Diffuse leptomeningeal glioneuronal tumour with hydrocephalus as an initial symptom: a case-based update. Child's Nerv Syst 36 (03) 459-468
    CrossrefPubMed
  • 5 Dyson K, Rivera-Zengotita M, Kresak J. et al. FGFR1 N546K and H3F3A K27M mutations in a diffuse leptomeningeal tumour with glial and neuronal markers. Histopathology 2016; 69 (04) 704-707
    CrossrefPubMedSearch in Google Scholar
  • 6 Karlowee V, Kolakshyapati M, Amatya VJ. et al. Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple's disease: a case report and literature review. Childs Nerv Syst 2017; 33 (08) 1411-1414
    CrossrefPubMedSearch in Google Scholar
  • 7 Tiwari N, Tamrazi B, Robison N, Krieger M, Ji J, Tian D. Unusual radiological and histological presentation of a diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 13-year-old girl. Childs Nerv Syst 2019; 35 (09) 1609-1614
    CrossrefPubMedSearch in Google Scholar
  • 8 Lee JK, Ko HC, Choi JG, Lee YS, Son BC. A case of diffuse leptomeningeal glioneuronal tumor misdiagnosed as chronic tuberculous meningitis without brain biopsy. Case Rep Neurol Med 2018; 2018: 1391943
    PubMedSearch in Google Scholar
  • 9 Yamasaki T, Sakai N, Shinmura K. et al. Anaplastic changes of diffuse leptomeningeal glioneuronal tumor with polar spongioblastoma pattern. Brain Tumor Pathol 2018; 35 (04) 209-216
    CrossrefPubMedSearch in Google Scholar
  • 10 Aguilera D, Castellino RC, Janss A. et al. Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy. Childs Nerv Syst 2018; 34 (02) 329-334
    CrossrefPubMedSearch in Google Scholar
  • 11 Nambirajan A, Suri V, Kedia S. et al. Paediatric diffuse leptomeningeal tumor with glial and neuronal differentiation harbouring chromosome 1p/19q co-deletion and H3.3 K27M mutation: unusual molecular profile and its therapeutic implications. Brain Tumor Pathol 2018; 35 (03) 186-191
    CrossrefPubMedSearch in Google Scholar
  • 12 Cho HJ, Myung JK, Kim H. et al. Primary diffuse leptomeningeal glioneuronal tumors. Brain Tumor Pathol 2015; 32 (01) 49-55
    CrossrefPubMedSearch in Google Scholar
  • 13 Schwetye KE, Kansagra AP, McEachern J, Schmidt RE, Gauvain K, Dahiya S. Unusual high-grade features in pediatric diffuse leptomeningeal glioneuronal tumor: comparison with a typical low-grade example. Hum Pathol 2017; 70: 105-112
    CrossrefPubMedSearch in Google Scholar
  • 14 Lyle MR, Dolia JN, Fratkin J, Nichols TA, Herrington BL. Newly identified characteristics and suggestions for diagnosis and treatment of diffuse leptomeningeal glioneuronal/neuroepithelial tumors: a case report and review of the literature. Child Neurol Open 2015; 2 (01) X14567531
    PubMedSearch in Google Scholar
  • 15 Xu H, Chen F, Zhu H, Luo L, Zhang R. Diffuse leptomeningeal glioneuronal tumor in a Chinese adult: a novel case report and review of literature. Acta Neurol Belg 2020; 120 (02) 247-256
    CrossrefPubMedSearch in Google Scholar
  • 16 Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A. High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol 2015; 129 (04) 609-610
    CrossrefPubMedSearch in Google Scholar
  • 17 Tiwari S, Yadav T, Pamnani J. et al. Diffuse leptomeningeal glioneuronal tumor: a unique leptomeningeal tumor entity. World Neurosurg 2020; 135: 297-300
    CrossrefPubMedSearch in Google Scholar
  • 18 Abongwa C, Cotter J, Tamrazi B, Dhall G, Davidson T, Margol A. Primary diffuse leptomeningeal glioneuronal tumors of the central nervous system: report of three cases and review of literature. Pediatr Hematol Oncol 2020; 37 (03) 248-258
    CrossrefPubMedSearch in Google Scholar