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Diffuse leptomeningeal glioneuronal tumour: where to biopsy? Case report and literature review

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Abstract

Purpose

Diffuse leptomeningeal glioneuronal tumour (DLGT) is an infrequent entity. Diagnosis is made with biopsy but with so few cases described management, prognosis remains undefined. There are not currently any articles regarding most effective place to biopsy.

Methods

Current literature review and introduction of the case of a 3-year-old male presenting at the emergency room with irritability, vomiting and nuchal rigidity. A head CT was made showing tetraventricular enlargement and a posterior fossa cyst.

Results

Patient underwent urgent ventriculoperitoneal shunting surgery with complete symptomp resolution. Brain MRI showed diffuse leptomeningeal enhancing, predominantly in basal cisterns, and multiple cystic-solid lesions along the neural axis. After ruling other conditions, a biopsy among intraoperative samples, was obtained of a PET positive gadolinium spinal enhancing lesion at D8 level, with the final diagnosis of DLGT.

Conclusion

DLGT can present as acute hydrocephalus. Biopsy stablishes the diagnosis but the place to take the sample can be difficult to select. Our experience suggests that PET-CT and intraoperative biopsy analysis can improve the effectivity of a representative sample.

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Authors and Affiliations

  1. Department of Paediatric Neurosurgery, Hospital La Paz, Paseo de la Castellana, 261, 28046, Madrid, Spain

    Miguel Sáez-Alegre, Javier Manuel Saceda Gutiérrez, Pablo García-Feijoo & Fernando Carceller Benito

  2. Department of Radiology, Hospital La Paz, Paseo de la Castellana, 261, 28046, Madrid, Spain

    Cristrina Utrilla Contreras

  3. Department of Paediatrics, Hospital La Paz, Paseo de la Castellana, 261, 28046, Madrid, Spain

    Francisco Javier Aracil Santos

Authors
  1. Miguel Sáez-Alegre
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  2. Javier Manuel Saceda Gutiérrez
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  3. Cristrina Utrilla Contreras
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  4. Francisco Javier Aracil Santos
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  5. Pablo García-Feijoo
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  6. Fernando Carceller Benito
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Correspondence to Miguel Sáez-Alegre.

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Sáez-Alegre, M., Saceda Gutiérrez, J.M., Utrilla Contreras, C. et al. Diffuse leptomeningeal glioneuronal tumour: where to biopsy? Case report and literature review. Childs Nerv Syst 37, 2405–2408 (2021). https://doi.org/10.1007/s00381-020-04955-2

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  • DOI: https://doi.org/10.1007/s00381-020-04955-2

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