Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update

Wenlin Chen, Ziren Kong, Ji Fu, Dachun Zhao, Renzhi Wang, Wenbin Ma, Yu Wang
Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery 2020, 36 (3): 459-468

PURPOSE: Diffuse leptomeningeal glioneuronal tumour (DLGNT) is a rare disease classified in 2016. There are different views of the clinical, pathologic and neuroradiologic characteristics of DLGNT due to the minor studies on this disease.

METHODS: We describe a case of a 12-year-old boy who initially presented intermittent headache, vomiting and communicating hydrocephalus. A literature review is also presented summarizing the clinical characteristics and treatments of DLGNT.

RESULTS: In our case, a ventriculoperitoneal shunt was applied to reduce intracranial pressure caused by communicating hydrocephalus. T1-weighted contrast-enhanced magnetic resonance imaging (MRI) showed linear enhancement, and microscopy showed tumour-like spindle cells. The diagnosis of DLGNT was confirmed, and temozolomide was administered. The clinical characteristics were similar in the reported cases, while the treatments showed differences.

CONCLUSION: Ventriculoperitoneal shunts are effective for patients with hydrocephalus-related intracranial hypertension. Chemotherapy including temozolomide has shown varying outcomes, and further studies are expected.

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