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Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: case report and review of the literature

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Abstract

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare central nervous system tumors of childhood that were recently described as a new entity. DLGNTs usually manifest with symptoms related to increased intracranial pressure or spinal cord compression. The classic radiological feature is a widespread leptomeningeal enhancement that may involve the entire neuroaxis. Microscopic examination demonstrates oligodendroglial-like cells that are positive for OLIG2, MAP2, and S100 and negative for IDH-1. Anaplastic features occur in some cases. Molecularly, DLGNTs are characterized by chromosome arm 1p deletion and alteration of a mitogen-activated protein kinase (MAPK) pathway gene, most commonly BRAF–KIAA1549 fusion. There is no established grading system for these tumors, which may have an indolent or aggressive behavior. Treatment usually involves chemotherapy and radiation therapy.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request. All data generated or analyzed during this study are included in this published article (and its supplementary information files).

Abbreviations

CNS:

Central nervous system

DLGNT:

Diffuse leptomeningeal glioneuronal tumor

MC:

Methylation class

MRI:

Magnetic resonance imaging

WHO:

World Health Organization

References

  1. Perry A, Capper D, Ellison DW et al (2021) Diffuse leptomeningeal glioneuronal tumour. In: WHO Classification of Tumours Editorial Board. World Health Organization Classification of Tumours of the Central Nervous System, 5th ed. Lyon. International Agency for Research on Cancer, Lyon, pp 139–142

  2. Lakhani DA, Mankad K, Chhabda S et al (2020) Leptomeningeal glioneuronal tumor of childhood. AJNR Am J Neuroradiol 41(11):2155–2159

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Deng MY, Sill M, Chiang J et al (2018) Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features. Acta Neuropathol 136(2):239–253

    Article  CAS  PubMed  Google Scholar 

  4. Karlowee V, Kolakshyapati M, Amatya VJ et al (2017) Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple’s disease: a case report and literature review. Childs Nerv Syst 33:1411–1414

    Article  PubMed  Google Scholar 

  5. Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A et al (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol 129(4):609–610

  6. Dodgshun AJ, SantaCruz N, Hwang J et al (2016) Disseminated glioneuronal tumors occurring in childhood: treatment outcomes and BRAF alterations including V600E mutation. J Neurooncol 128(2):293–302

    Article  CAS  PubMed  Google Scholar 

  7. Abongwa C, Cotter J, Tamrazi B et al (2020) Primary diffuse leptomeningeal glioneuronal tumors of the central nervous system: report of three cases and review of literature. Pediatr Hematol Oncol 37(3):248–258

    Article  PubMed  Google Scholar 

  8. Sarkinaite M, Devyziene I, Makstiene J et al (2022) Diffuse leptomeningeal glioneuronal tumour with 9-year follow-up: case report and review of the literature. Diagnostics 12(2):342

    Article  PubMed  PubMed Central  Google Scholar 

  9. Schwetye KE, Kansagra AP, McEachern J et al (2017) Unusual high-grade features in pediatric diffuse leptomeningeal glioneuronal tumor: comparison with a typical low-grade example. Hum Pathol 70:105–112

    Article  PubMed  Google Scholar 

  10. Aguilera D, Castellino RC, Janss A et al (2018) Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy. Childs Nerv Syst 34(2):329–334

    Article  PubMed  Google Scholar 

  11. Lyle MR, Dolia JN, Fratkin J et al (2015) 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 2(1):2329048X14567531

  12. Manoharan N, Ajuyah P, Senapati A et al (2021) Diffuse leptomeningeal glioneuronal tumour (DLGNT) in children: the emerging role of genomic analysis. Acta Neuropathol Commun 9(1):147

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Author information

Authors and Affiliations

  1. Federal University of Rio Grande Do Sul, Porto Alegre, Rio Grande do Sul, Brazil

    Eduardo Cambruzzi

  2. Department of Pathology, Santa Rita Hospital, Santa Casa Hospital Complex, Rua Sarmento Leite, 187, 2° andar, Porto Alegre, Rio Grande do Sul, Brazil

    Eduardo Cambruzzi

  3. Conceição Hospital Group, Porto Alegre, Rio Grande do Sul, Brazil

    Eduardo Cambruzzi, Mateus Scarabelot Medeiros, Carmo Eduardo Cardoso, Guilherme Alberto Germano Silva, Kelly Schlotte & Willian Pegoraro Kus

  4. Institute of Cardiology, University Foundation of Cardiology, Porto Alegre, Rio Grande do Sul, Brazil

    Eduardo Cambruzzi

  5. Unisinos, Faculty of Medicine, Rio Grande do Sul, São Leopoldo, Brazil

    Eduardo Cambruzzi

Authors
  1. Eduardo Cambruzzi
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  2. Mateus Scarabelot Medeiros
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  3. Carmo Eduardo Cardoso
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  4. Guilherme Alberto Germano Silva
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  5. Kelly Schlotte
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  6. Willian Pegoraro Kus
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Contributions

Eduardo Cambruzzi: work design, scientific review, writing the manuscript. Mateus Scarabelot Medeiros: work design, scientific review. Carmo Eduardo Cardoso: data collect. Guilherme Alberto Germano Silva: data collect. Kelly Schlotte: data collect. Willian Pegoraro Kus: prepared figures.

Corresponding author

Correspondence to Eduardo Cambruzzi.

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Ethics approval

Approval was obtained from the ethics committee of Hospital Nossa Senhora da Conceição, Porto Alegre, RS, Brazil. The procedures used in this study adhere to the tenets of the Declaration of Helsinki.

Conflict of interest

The authors declare no conflict of interest.

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Case article

In this article, we report a case of DLGNT in an 8-year-old girl with symptoms related to spinal cord compression as the initial manifestation.

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Cambruzzi, E., Medeiros, M.S., Cardoso, C.E. et al. Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: case report and review of the literature. Childs Nerv Syst 39, 301–305 (2023). https://doi.org/10.1007/s00381-022-05625-1

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  • DOI: https://doi.org/10.1007/s00381-022-05625-1

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