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

Childs Nerv Syst. 2021 Jul;37(7):2405-2408. doi: 10.1007/s00381-020-04955-2. Epub 2020 Oct 31.

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.

Keywords: Diffuse leptomeningeal glioneuronal tumor; Neurosurgery; Paediatrics.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Biopsy
  • Brain Neoplasms*
  • Child, Preschool
  • Humans
  • Male
  • Meningeal Neoplasms* / diagnostic imaging
  • Meningeal Neoplasms* / surgery
  • Meninges
  • Positron Emission Tomography Computed Tomography