AUTHOR=Chen Wenlin , Jin Shanmu , Liu Qianshu , Wang Hai , Xia Yu , Guo Xiaopeng , Guo Siying , Wang Yaning , Shi Yixin , Liu Delin , Li Yilin , Wang Yuekun , Xing Hao , Li Junlin , Wu Jiaming , Liang Tingyu , Qu Tian , Li Huanzhang , Yang Tianrui , Zhang Kun , Wang Yu , Ma Wenbin 

TITLE=Spotlights on adult patients with pediatric-type diffuse gliomas in accordance with the 2021 WHO classification of CNS tumors

JOURNAL=Frontiers in Neuroscience

VOLUME=17

YEAR=2023

URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2023.1144559

DOI=10.3389/fnins.2023.1144559

ISSN=1662-453X

ABSTRACT=<sec><title>Introduction</title><p>The fifth edition of the World Health Organization (WHO) classification of central nervous system (CNS) tumors released in 2021 formally defines pediatric-type diffuse gliomas. However, there is still little understanding of pediatric-type diffuse gliomas, and even less attention has been paid to adult patients. Therefore, this study describes the clinical radiological, survival, and molecular features of adult patients with pediatric-type glioma.</p></sec><sec><title>Methods</title><p>Adult patients who underwent surgery from January 2011 to January 2022, classified as pediatric-type glioma, were included in this study. Clinical, radiological, histopathological, molecular pathological, and survival data were collected for analysis.</p></sec><sec><title>Results</title><p>Among 596 adult patients, 20 patients with pediatric-type glioma were screened, including 6 with diffuse astrocytoma, <italic>MYB</italic>- or <italic>MYBL1</italic>-altered, 2 with diffuse midline glioma, H3 K27-altered, and 12 with diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype. Pediatric high-grade glioma (pHGG) frequently showed tumor enhancement, peritumoral edema, and intratumoral necrosis. Adult patients with pHGG showed a longer life expectancy than adult patients with glioblastoma. Common molecular alterations included chromosome alterations and <italic>CDKN2A/B</italic>, <italic>PIK3CA</italic>, and <italic>PTEN</italic>, while altered <italic>KMT5B</italic> and <italic>MET</italic> were found to affect the overall survival.</p></sec><sec><title>Conclusion</title><p>Our study demonstrated adult patients with pediatric-type glioma. Notably, our research aims to expand the current understanding of adult patients with pediatric-type diffuse gliomas. Furthermore, personalized therapies consisting of targeted molecular inhibitors for <italic>MET</italic> and <italic>VEGFA</italic> may exhibit beneficial effects in the corresponding population.</p></sec>