Clinical progression, pathological characteristics, and radiological findings in children with diffuse leptomeningeal glioneuronal tumors: A systematic review

• Published in: Frontiers in oncology 2022-09, Vol.12, p.970076-970076
• Main Authors: Jiang, Haoxiang, Qiu, Lu, Song, Juan, Xu, Dandan, Sun, Lei, Feng, Yinbo, Zhao, Jun, Qian, Jun, Yu, Zhiwei, Peng, Jin
• Format: Article
• Language: English
• Subjects: clinical progression; Diffuse Leptomeningeal Glioneuronal Tumor; Oncology; pathology; pediatrics; radiology; systematic review
• ISSN: 2234-943X; 2234-943X
• DOI: 10.3389/fonc.2022.970076

BackgroundDiffuse leptomeningeal glioneuronal tumors are rare leptomeningeal neoplasms composed of oligodendrocyte-like cells characterized by neuronal differentiation and a lack of isocitrate dehydrogenase gene mutation. PurposeWe aimed to analyze the clinical progression, pathological characteristics, and radiological findings of diffuse leptomeningeal glioneuronal tumors in children, as well as the relevance of clinico-radiological data. Data SourcesWe searched MEDLINE, PubMed, and Web of Science to identify case reports, original articles, and review articles discussing diffuse leptomeningeal glioneuronal tumors published between 2000 and 2021. Study SelectionThe analysis included 145 pediatric patients from 43 previous studies. Data AnalysisData regarding patient pathology, MRI manifestations, clinical symptoms, and progression were collected. The relationship between imaging classification and pathological findings was using chi-square tests. Overall survival was analyzed using Kaplan-Meier curves. Data SynthesisParenchymal tumors were mainly located in the intramedullary areas of the cervical and thoracic spine, and patients which such tumors were prone to 1p-deletion (χ2 = 4.77, p=0.03) and KIAA1549-BRAF fusion (χ2 = 12.17, p