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

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.