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Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update
Wenlin Chen1, Ziren Kong1, Ji Fu1
1Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, China.
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.
