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Review
. 2023 Jan;39(1):301-305.
doi: 10.1007/s00381-022-05625-1. Epub 2022 Aug 3.

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

Eduardo Cambruzzi  1   2   3   4   5 , Mateus Scarabelot Medeiros  6 , Carmo Eduardo Cardoso  6 , Guilherme Alberto Germano Silva  6 , Kelly Schlotte  6 , Willian Pegoraro Kus  6
Affiliations
Review

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

Eduardo Cambruzzi et al. Childs Nerv Syst. 2023 Jan.

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.

Keywords: Diffuse leptomeningeal glioneuronal tumor; Leptomeningeal neoplasms; Neurosurgery; Pediatrics.

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References

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