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.
Keywords: Diffuse leptomeningeal enhancement; Diffuse leptomeningeal glioneuronal tumour; Disseminated oligodendroglial–like leptomeningeal tumour; Hydrocephalus.