Abstract

Chordoid glioma (CG), a rare slow-growing brain tumor, mainly occurs in the region of the third ventricle. Although its degree of malignancy is relatively low, its clinical prognosis is poor due to obscure clinical manifestations and the particular growing position. Currently, gross total resection is the best available method for treatment of CG. However, the tumor is located in the deep structure of the brain and close to neurovascular structure so it is difficult to remove completely. This study reported a case of CG of the third ventricle 5 years after surgery of right frontal parietal fibrous meningioma, accompanied with peri and post-operative sodium ion metabolism disorder. Whole-exome sequencing (WES) revealed 25 gene mutations shared by meningioma and CG. In addition, the PRKCAD463H CG marker gene mutation also existed in this patient. We reviewed the latest literature on this rare brain tumor, summarized its clinical manifestations, imaging and pathological characteristics, and discussed the mechanism related to its occurrence and the reasons for sodium ion disorder.

Highlights

  • Fibrous meningioma and chordoid glioma (CG), two primary intracranial tumors of different origins, have disparate pathological manifestations

  • We reported a case of third ventricle CG, which occurred 5 years after the total resection of fibrous meningioma, and the peri and postoperative ion disorder may be related to endocrine metabolic diseases

  • We reviewed the clinical manifestations, imaging, and pathological features of the tumor, and focused on the pathogenesis of the tumor and causes of abnormal sodium ion metabolism to deepen the clinical understanding of CG

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Summary

INTRODUCTION

Fibrous meningioma and chordoid glioma (CG), two primary intracranial tumors of different origins, have disparate pathological manifestations. We reported a case of third ventricle CG, which occurred 5 years after the total resection of fibrous meningioma, and the peri and postoperative ion disorder may be related to endocrine metabolic diseases. The tumor was completely resected microsurgically through the right frontal top approach, and a post-operative pathological diagnosis of meningioma was reached (fibrous, WHO I) (Figures 1A,B). Consistent with previously reported cases, the meningioma in this patient harbored a mutation on chromosome 16. 17, a G>C transversion mutation in the protein kinase C alpha (PRKCA) gene was identified, causing a c.1387G>C, p.D463H substitution (reference transcript NM_002737.2) (Figure 2). In Tier-Based reporting, it is classified as Tier III Variants: Variants of Unknown Significance

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